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Автор: Grafiati
Опубліковано: 6 червня 2023

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1

Kirby, Alastair J., Thomas Palmer, Richard J. Mead, Ronaldo M. Ichiyama, and Samit Chakrabarty. "Caudal–Rostral Progression of Alpha Motoneuron Degeneration in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis." Antioxidants 11, no. 5 (May 17, 2022): 983. http://dx.doi.org/10.3390/antiox11050983.

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Mice with transgenic expression of human SOD1G93A are a widely used model of ALS, with a caudal–rostral progression of motor impairment. Previous studies have quantified the progression of motoneuron (MN) degeneration based on size, even though alpha (α-) and gamma (γ-) MNs overlap in size. Therefore, using molecular markers and synaptic inputs, we quantified the survival of α-MNs and γ-MNs at the lumbar and cervical spinal segments of 3- and 4-month SOD1G93A mice, to investigate whether there is a caudal–rostral progression of MN death. By 3 months, in the cervical and lumbar spinal cord, there was α-MN degeneration with complete γ-MN sparing. At 3 months, the cervical spinal cord had more α-MNs per ventral horn than the lumbar spinal cord in SOD1G93A mice. A similar spatial trend of degeneration was observed in the corticospinal tract, which remained intact in the cervical spinal cord at 3- and 4- months of age. These findings agree with the corticofugal synaptopathy model that α-MNs and CST of the lumbar spinal cord are more susceptible to degeneration in SOD1G93A mice. Hence, there is a spatial and temporal caudal–rostral progression of α-MN and CST degeneration in SOD1G93A mice.
2

Bonifacino, Tiziana, Claudia Rebosio, Francesca Provenzano, Carola Torazza, Matilde Balbi, Marco Milanese, Luca Raiteri, Cesare Usai, Ernesto Fedele, and Giambattista Bonanno. "Enhanced Function and Overexpression of Metabotropic Glutamate Receptors 1 and 5 in the Spinal Cord of the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis during Disease Progression." International Journal of Molecular Sciences 20, no. 18 (September 13, 2019): 4552. http://dx.doi.org/10.3390/ijms20184552.

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Glutamate (Glu)-mediated excitotoxicity is a major cause of amyotrophic lateral sclerosis (ALS) and our previous work highlighted that abnormal Glu release may represent a leading mechanism for excessive synaptic Glu. We demonstrated that group I metabotropic Glu receptors (mGluR1, mGluR5) produced abnormal Glu release in SOD1G93A mouse spinal cord at a late disease stage (120 days). Here, we studied this phenomenon in pre-symptomatic (30 and 60 days) and early-symptomatic (90 days) SOD1G93A mice. The mGluR1/5 agonist (S)-3,5-Dihydroxyphenylglycine (3,5-DHPG) concentration dependently stimulated the release of [3H]d-Aspartate ([3H]d-Asp), which was comparable in 30- and 60-day-old wild type mice and SOD1G93A mice. At variance, [3H]d-Asp release was significantly augmented in 90-day-old SOD1G93A mice and both mGluR1 and mGluR5 were involved. The 3,5-DHPG-induced [3H]d-Asp release was exocytotic, being of vesicular origin and mediated by intra-terminal Ca2+ release. mGluR1 and mGluR5 expression was increased in Glu spinal cord axon terminals of 90-day-old SOD1G93A mice, but not in the whole axon terminal population. Interestingly, mGluR1 and mGluR5 were significantly augmented in total spinal cord tissue already at 60 days. Thus, function and expression of group I mGluRs are enhanced in the early-symptomatic SOD1G93A mouse spinal cord, possibly participating in excessive Glu transmission and supporting their implication in ALS. Please define all abbreviations the first time they appear in the abstract, the main text, and the first figure or table caption.
3

Martin, Elodie, William Cazenave, Anne-Emilie Allain, Daniel Cattaert, and Pascal Branchereau. "Implication of 5-HT in the Dysregulation of Chloride Homeostasis in Prenatal Spinal Motoneurons from the G93A Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 21, no. 3 (February 7, 2020): 1107. http://dx.doi.org/10.3390/ijms21031107.

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Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive motor neuron degeneration and muscle paralysis. The early presymptomatic onset of abnormal processes is indicative of cumulative defects that ultimately lead to a late manifestation of clinical symptoms. It remains of paramount importance to identify the primary defects that underlie this condition and to determine how these deficits lead to a cycle of deterioration. We recently demonstrated that prenatal E17.5 lumbar spinal motoneurons (MNs) from SOD1G93A mice exhibit a KCC2-related alteration in chloride homeostasis, i.e., the EGABAAR is more depolarized than in WT littermates. Here, using immunohistochemistry, we found that the SOD1G93A lumbar spinal cord is less enriched with 5-HT descending fibres than the WT lumbar spinal cord. High-performance liquid chromatography confirmed the lower level of the monoamine 5-HT in the SOD1G93A spinal cord compared to the WT spinal cord. Using ex vivo perforated patch-clamp recordings of lumbar MNs coupled with pharmacology, we demonstrated that 5-HT strongly hyperpolarizes the EGABAAR by interacting with KCC2. Therefore, the deregulation of the interplay between 5-HT and KCC2 may explain the alteration in chloride homeostasis detected in prenatal SOD1G93A MNs. In conclusion, 5-HT and KCC2 are two likely key factors in the presymptomatic phase of ALS, particular in familial ALS involving the SOD1G93A mutation.
4

Rudnick, Noam D., Christopher J. Griffey, Paolo Guarnieri, Valeria Gerbino, Xueyong Wang, Jason A. Piersaint, Juan Carlos Tapia, Mark M. Rich, and Tom Maniatis. "Distinct roles for motor neuron autophagy early and late in the SOD1G93A mouse model of ALS." Proceedings of the National Academy of Sciences 114, no. 39 (September 13, 2017): E8294—E8303. http://dx.doi.org/10.1073/pnas.1704294114.

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Mutations in autophagy genes can cause familial and sporadic amyotrophic lateral sclerosis (ALS). However, the role of autophagy in ALS pathogenesis is poorly understood, in part due to the lack of cell type-specific manipulations of this pathway in animal models. Using a mouse model of ALS expressing mutant superoxide dismutase 1 (SOD1G93A), we show that motor neurons form large autophagosomes containing ubiquitinated aggregates early in disease progression. To investigate whether this response is protective or detrimental, we generated mice in which the critical autophagy gene Atg7 was specifically disrupted in motor neurons (Atg7 cKO). Atg7 cKO mice were viable but exhibited structural and functional defects at a subset of vulnerable neuromuscular junctions. By crossing Atg7 cKO mice to the SOD1G93A mouse model, we found that autophagy inhibition accelerated early neuromuscular denervation of the tibialis anterior muscle and the onset of hindlimb tremor. Surprisingly, however, lifespan was extended in Atg7 cKO; SOD1G93A double-mutant mice. Autophagy inhibition did not prevent motor neuron cell death, but it reduced glial inflammation and blocked activation of the stress-related transcription factor c-Jun in spinal interneurons. We conclude that motor neuron autophagy is required to maintain neuromuscular innervation early in disease but eventually acts in a non–cell-autonomous manner to promote disease progression.
5

Chiu, Isaac M., Adam Chen, Yi Zheng, Bela Kosaras, Stefanos A. Tsiftsoglou, Timothy K. Vartanian, Robert H. Brown, and Michael C. Carroll. "T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS." Proceedings of the National Academy of Sciences 105, no. 46 (November 7, 2008): 17913–18. http://dx.doi.org/10.1073/pnas.0804610105.

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Amyotrophic Lateral Sclerosis (ALS) is an adult-onset, progressive, motor neuron degenerative disease, in which the role of inflammation is not well established. Innate and adaptive immunity were investigated in the CNS of the Superoxide Dismutase 1 (SOD1)G93A transgenic mouse model of ALS. CD4+ and CD8+ T cells infiltrated SOD1G93A spinal cords during disease progression. Cell-specific flow cytometry and gene expression profiling showed significant phenotypic changes in microglia, including dendritic cell receptor acquisition, and expression of genes linked to neuroprotection, cholesterol metabolism and tissue remodeling. Microglia dramatically up-regulated IGF-1 and down-regulated IL-6 expression. When mutant SOD1 mice were bred onto a TCRβ deficient background, disease progression was significantly accelerated at the symptomatic stage. In addition, microglia reactivity and IGF-1 levels were reduced in spinal cords of SOD1G93A (TCRβ−/−) mice. These results indicate that T cells play an endogenous neuroprotective role in ALS by modulating a beneficial inflammatory response to neuronal injury.
6

Tankersley, Clarke G., Christine Haenggeli, and Jeffery D. Rothstein. "Respiratory impairment in a mouse model of amyotrophic lateral sclerosis." Journal of Applied Physiology 102, no. 3 (March 2007): 926–32. http://dx.doi.org/10.1152/japplphysiol.00193.2006.

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Amyothrophic lateral sclerosis (ALS) is a progressive, lethal neuromuscular disease that is associated with the degeneration of cortical and spinal motoneurons, leading to atrophy of limb, axial, and respiratory muscles. Patients with ALS invariably develop respiratory muscle weakness and most die from pulmonary complications. Overexpression of superoxide dismutase 1 ( SOD1) gene mutations in mice recapitulates several of the clinical and pathological characteristics of ALS and is therefore a valuable tool to study this disease. The present study is intended to evaluate an age-dependent progression of respiratory complications in SOD1G93A mutant mice. In each animal, baseline measurements of breathing pattern [i.e., breathing frequency and tidal volume (Vt)], minute ventilation (Ve), and metabolism (i.e., oxygen consumption and carbon dioxide production) were repeatedly sampled at variable time points between 10 and 20 wk of age with the use of whole-body plethysmographic chambers. To further characterize the neurodegeneration of breathing, Ve was also measured during 5-min challenges of hypercapnia (5% CO2) and hypoxia (10% O2). At baseline, breathing characteristics and metabolism remained relatively unchanged from 10 to 14 wk of age. From 14 to 18 wk of age, there were significant ( P < 0.05) increases in baseline Vt, Ve, and the ventilatory equivalent (Ve/oxygen consumption). After 18 wk of age, there was a rapid decline in Ve due to significant ( P < 0.05) reductions in both breathing frequency and Vt. Whereas little change in hypoxic Ve responses occurred between 10 and 18 wk, hypercapnic Ve responses were significantly ( P < 0.05) elevated at 18 wk due to an augmented Vt response. Like baseline breathing characteristics, hypercapnic Ve responses also declined rapidly after 18 wk of age. The phenotypic profile of SOD1G93A mutant mice was apparently unique because similar changes in respiration and metabolism were not observed in SOD1 controls. The present results outline the magnitude and time course of respiratory complications in SOD1G93A mutant mice as the progression of disease occurs in this mouse model of ALS.
7

Moreno-Martinez, Laura, Miriam de la Torre, María J. Muñoz, Pilar Zaragoza, José Aguilera, Ana C. Calvo, and Rosario Osta. "Neuroprotective Fragment C of Tetanus Toxin Modulates IL-6 in an ALS Mouse Model." Toxins 12, no. 5 (May 17, 2020): 330. http://dx.doi.org/10.3390/toxins12050330.

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Neuroinflammation plays a significant role in amyotrophic lateral sclerosis (ALS) pathology, leading to the development of therapies targeting inflammation in recent years. Our group has studied the tetanus toxin C-terminal fragment (TTC) as a therapeutic molecule, showing neuroprotective properties in the SOD1G93A mouse model. However, it is unknown whether TTC could have some effect on inflammation. The objective of this study was to assess the effect of TTC on the regulation of inflammatory mediators to elucidate its potential role in modulating inflammation occurring in ALS. After TTC treatment in SOD1G93A mice, levels of eotaxin-1, interleukin (IL)-2, IL-6 and macrophage inflammatory protein (MIP)-1 alpha (α) and galectin-1 were analyzed by immunoassays in plasma samples, whilst protein expression of caspase-1, IL-1β, IL-6 and NOD-, LRR- and pyrin domain-containing protein 3 (NLRP3) was measured in the spinal cord, extensor digitorum longus (EDL) muscle and soleus (SOL) muscle. The results showed reduced levels of IL-6 in spinal cord, EDL and SOL in treated SOD1G93A mice. In addition, TTC showed a different role in the modulation of NLRP3 and caspase-1 depending on the tissue analyzed. In conclusion, our results suggest that TTC could have a potential anti-inflammatory effect by reducing IL-6 levels in tissues drastically affected by the disease. However, further research is needed to study more in depth the anti-inflammatory effect of TTC in ALS.
8

Rojas, Pilar, Ana I. Ramírez, Manuel Cadena, José A. Fernández-Albarral, Elena Salobrar-García, Inés López-Cuenca, Irene Santos-García, et al. "Retinal Ganglion Cell Loss and Microglial Activation in a SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 22, no. 4 (February 7, 2021): 1663. http://dx.doi.org/10.3390/ijms22041663.

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The neurodegenerative disease amyotrophic lateral sclerosis (ALS) affects the spinal cord, brain stem, and cerebral cortex. In this pathology, both neurons and glial cells are affected. However, few studies have analyzed retinal microglia in ALS models. In this study, we quantified the signs of microglial activation and the number of retinal ganglion cells (RGCs) in an SOD1G93A transgenic mouse model at 120 days (advanced stage of the disease) in retinal whole-mounts. For SOD1G93A animals (compared to the wild-type), we found, in microglial cells, (i) a significant increase in the area occupied by each microglial cell in the total area of the retina; (ii) a significant increase in the arbor area in the outer plexiform layer (OPL) inferior sector; (iii) the presence of cells with retracted processes; (iv) areas of cell groupings in some sectors; (v) no significant increase in the number of microglial cells; (vi) the expression of IFN-γ and IL-1β; and (vii) the non-expression of IL-10 and arginase-I. For the RGCs, we found a decrease in their number. In conclusion, in the SOD1G93A model (at 120 days), retinal microglial activation occurred, taking a pro-inflammatory phenotype M1, which affected the OPL and inner retinal layers and could be related to RGC loss.
9

Mallozzi, Cinzia, Alida Spalloni, Patrizia Longone, and Maria Rosaria Domenici. "Activation of Phosphotyrosine-Mediated Signaling Pathways in the Cortex and Spinal Cord of SOD1G93A, a Mouse Model of Familial Amyotrophic Lateral Sclerosis." Neural Plasticity 2018 (August 5, 2018): 1–10. http://dx.doi.org/10.1155/2018/2430193.

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Degeneration of cortical and spinal motor neurons is the typical feature of amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease for which a pathogenetic role for the Cu/Zn superoxide dismutase (SOD1) has been demonstrated. Mice overexpressing a mutated form of the SOD1 gene (SOD1G93A) develop a syndrome that closely resembles the human disease. The SOD1 mutations confer to this enzyme a “gain-of-function,” leading to increased production of reactive oxygen species. Several oxidants induce tyrosine phosphorylation through direct stimulation of kinases and/or phosphatases. In this study, we analyzed the activities of src and fyn tyrosine kinases and of protein tyrosine phosphatases in synaptosomal fractions prepared from the motor cortex and spinal cord of transgenic mice expressing SOD1G93A. We found that (i) protein phosphotyrosine level is increased, (ii) src and fyn activities are upregulated, and (iii) the activity of tyrosine phosphatases, including the striatal-enriched tyrosine phosphatase (STEP), is significantly decreased. Moreover, the NMDA receptor (NMDAR) subunit GluN2B tyrosine phosphorylation was upregulated in SOD1G93A. Tyrosine phosphorylation of GluN2B subunits regulates the NMDAR function and the recruitment of downstream signaling molecules. Indeed, we found that proline-rich tyrosine kinase 2 (Pyk2) and ERK1/2 kinase are upregulated in SOD1G93A mice. These results point out an involvement of tyrosine kinases and phosphatases in the pathogenesis of ALS.
10

Rei, Nádia, Cláudia A. Valente, Sandra H. Vaz, Miguel Farinha-Ferreira, Joaquim A. Ribeiro, and Ana M. Sebastião. "Changes in adenosine receptors and neurotrophic factors in the SOD1G93A mouse model of amyotrophic lateral sclerosis: Modulation by chronic caffeine." PLOS ONE 17, no. 12 (December 14, 2022): e0272104. http://dx.doi.org/10.1371/journal.pone.0272104.

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Amyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of corticospinal tract motor neurons. Previous studies showed that adenosine-mediated neuromodulation is disturbed in ALS and that vascular endothelial growth factor (VEGF) has a neuroprotective function in ALS mouse models. We evaluated how adenosine (A1R and A2AR) and VEGF (VEGFA, VEGFB, VEGFR-1 and VEGFR-2) system markers are altered in the cortex and spinal cord of pre-symptomatic and symptomatic SOD1G93A mice. We then assessed if/how chronic treatment of SOD1G93A mice with a widely consumed adenosine receptor antagonist, caffeine, modulates VEGF system and/or the levels of Brain-derived Neurotrophic Factor (BDNF), known to be under control of A2AR. We found out decreases in A1R and increases in A2AR levels even before disease onset. Concerning the VEGF system, we detected increases of VEGFB and VEGFR-2 levels in the spinal cord at pre-symptomatic stage, which reverses at the symptomatic stage, and decreases of VEGFA levels in the cortex, in very late disease states. Chronic treatment with caffeine rescued cortical A1R levels in SOD1G93A mice, bringing them to control levels, while rendering VEGF signaling nearly unaffected. In contrast, BDNF levels were significantly affected in SOD1G93A mice treated with caffeine, being decreased in the cortex and increased in spinal the cord. Altogether, these findings suggest an early dysfunction of the adenosinergic system in ALS and highlights the possibility that the negative influence of caffeine previously reported in ALS animal models results from interference with BDNF rather than with the VEGF signaling molecules.
11

Post, Julia, Anja Schaffrath, Ian Gering, Sonja Hartwig, Stefan Lehr, N. Jon Shah, Karl-Josef Langen, Dieter Willbold, Janine Kutzsche, and Antje Willuweit. "Oral Treatment with RD2RD2 Impedes Development of Motoric Phenotype and Delays Symptom Onset in SOD1G93A Transgenic Mice." International Journal of Molecular Sciences 22, no. 13 (June 30, 2021): 7066. http://dx.doi.org/10.3390/ijms22137066.

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Neuroinflammation is a pathological hallmark of several neurodegenerative disorders and plays a key role in the pathogenesis of amyotrophic lateral sclerosis (ALS). It has been implicated as driver of disease progression and is observed in ALS patients, as well as in the transgenic SOD1G93A mouse model. Here, we explore and validate the therapeutic potential of the d-enantiomeric peptide RD2RD2 upon oral administration in SOD1G93A mice. Transgenic mice were treated daily with RD2RD2 or placebo for 10 weeks and phenotype progression was followed with several behavioural tests. At the end of the study, plasma cytokine levels and glia cell markers in brain and spinal cord were analysed. Treatment resulted in a significantly increased performance in behavioural and motor coordination tests and a decelerated neurodegenerative phenotype in RD2RD2-treated SOD1G93A mice. Additionally, we observed retardation of the average disease onset. Treatment of SOD1G93A mice led to significant reduction in glial cell activation and a rescue of neurons. Analysis of plasma revealed normalisation of several cytokines in samples of RD2RD2-treated SOD1G93A mice towards the levels of non-transgenic mice. In conclusion, these findings qualify RD2RD2 to be considered for further development and testing towards a disease modifying ALS treatment.
12

Bette, Michael, Eileen Cors, Carolin Kresse, and Burkhard Schütz. "Therapeutic Treatment of Superoxide Dismutase 1 (G93A) Amyotrophic Lateral Sclerosis Model Mice with Medical Ozone Decelerates Trigeminal Motor Neuron Degeneration, Attenuates Microglial Proliferation, and Preserves Monocyte Levels in Mesenteric Lymph Nodes." International Journal of Molecular Sciences 23, no. 6 (March 21, 2022): 3403. http://dx.doi.org/10.3390/ijms23063403.

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Amyotrophic lateral sclerosis (ALS) is an incurable and lethal neurodegenerative disease in which progressive motor neuron loss and associated inflammation represent major pathology hallmarks. Both the prevention of neuronal loss and neuro-destructive inflammation are still unmet challenges. Medical ozone, an ozonized oxygen mixture (O3/O2), has been shown to elicit profound immunomodulatory effects in peripheral organs, and beneficial effects in the aging brain. We investigated, in a preclinical drug testing approach, the therapeutic potential of a five-day O3/O2i.p. treatment regime at the beginning of the symptomatic disease phase in the superoxide dismutase (SOD1G93A) ALS mouse model. Clinical assessment of SOD1G93A mice revealed no benefit of medical ozone treatment over sham with respect to gross body weight, motor performance, disease duration, or survival. In the brainstem of end stage SOD1G93A mice, however, neurodegeneration was found decelerated, and SOD1-related vacuolization was reduced in the motor trigeminal nucleus in the O3/O2 treatment group when compared to sham-treated mice. In addition, microglia proliferation was less pronounced in the brainstem, while the hypertrophy of astroglia remained largely unaffected. Finally, monocyte numbers were reduced in the blood, spleen, and mesenteric lymph nodes at postnatal day 60 in SOD1G93A mice. A further decrease in monocyte numbers seen in mesenteric lymph nodes from sham-treated SOD1G93A mice at an advanced disease stage, however, was prevented by medical ozone treatment. Collectively, our study revealed a select neuroprotective and possibly anti-inflammatory capacity for medical ozone when applied as a therapeutic agent in SOD1G93A ALS mice.
13

Crivello, Martin, Saidhbhe L. O'Riordan, Ina Woods, Sarah Cannon, Luise Halang, Karen S. Coughlan, Marion C. Hogg, Sebastian A. Lewandowski, and Jochen H. M. Prehn. "Pleiotropic activity of systemically delivered angiogenin in the SOD1G93A mouse model." Neuropharmacology 133 (May 2018): 503–11. http://dx.doi.org/10.1016/j.neuropharm.2018.02.022.

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14

Bame, Monica, Robert E. Grier, Richard Needleman, and William S. A. Brusilow. "Amino Acids as biomarkers in the SOD1G93A mouse model of ALS." Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1842, no. 1 (January 2014): 79–87. http://dx.doi.org/10.1016/j.bbadis.2013.10.004.

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15

Marini, Cecilia, Vanessa Cossu, Mandeep Kumar, Marco Milanese, Katia Cortese, Silvia Bruno, Grazia Bellese, et al. "The Role of Endoplasmic Reticulum in the Differential Endurance against Redox Stress in Cortical and Spinal Astrocytes from the Newborn SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis." Antioxidants 10, no. 9 (August 30, 2021): 1392. http://dx.doi.org/10.3390/antiox10091392.

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Recent studies reported that the uptake of [18F]-fluorodeoxyglucose (FDG) is increased in the spinal cord (SC) and decreased in the motor cortex (MC) of patients with ALS, suggesting that the disease might differently affect the two nervous districts with different time sequence or with different mechanisms. Here we show that MC and SC astrocytes harvested from newborn B6SJL-Tg (SOD1G93A) 1Gur mice could play different roles in the pathogenesis of the disease. Spectrophotometric and cytofluorimetric analyses showed an increase in redox stress, a decrease in antioxidant capacity and a relative mitochondria respiratory uncoupling in MC SOD1G93A astrocytes. By contrast, SC mutated cells showed a higher endurance against oxidative damage, through the increase in antioxidant defense, and a preserved respiratory function. FDG uptake reproduced the metabolic response observed in ALS patients: SOD1G93A mutation caused a selective enhancement in tracer retention only in mutated SC astrocytes, matching the activity of the reticular pentose phosphate pathway and, thus, of hexose-6P dehydrogenase. Finally, both MC and SC mutated astrocytes were characterized by an impressive ultrastructural enlargement of the endoplasmic reticulum (ER) and impairment in ER–mitochondria networking, more evident in mutated MC than in SC cells. Thus, SOD1G93A mutation differently impaired MC and SC astrocyte biology in a very early stage of life.
16

Dobrowolny, Gabriella, Cristina Giacinti, Laura Pelosi, Carmine Nicoletti, Nadine Winn, Laura Barberi, Mario Molinaro, Nadia Rosenthal, and Antonio Musarò. "Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model." Journal of Cell Biology 168, no. 2 (January 17, 2005): 193–99. http://dx.doi.org/10.1083/jcb.200407021.

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by a selective degeneration of motor neurons, atrophy, and paralysis of skeletal muscle. Although a significant proportion of familial ALS results from a toxic gain of function associated with dominant SOD1 mutations, the etiology of the disease and its specific cellular origins have remained difficult to define. Here, we show that muscle-restricted expression of a localized insulin-like growth factor (Igf) -1 isoform maintained muscle integrity and enhanced satellite cell activity in SOD1G93A transgenic mice, inducing calcineurin-mediated regenerative pathways. Muscle-specific expression of local Igf-1 (mIgf-1) isoform also stabilized neuromuscular junctions, reduced inflammation in the spinal cord, and enhanced motor neuronal survival in SOD1G93A mice, delaying the onset and progression of the disease. These studies establish skeletal muscle as a primary target for the dominant action of inherited SOD1 mutation and suggest that muscle fibers provide appropriate factors, such as mIgf-1, for neuron survival.
17

Varghese, Merina, Wei Zhao, Kyle J. Trageser та Giulio M. Pasinetti. "Peroxisome Proliferator Activator Receptor Gamma Coactivator-1α Overexpression in Amyotrophic Lateral Sclerosis: A Tale of Two Transgenics". Biomolecules 10, № 5 (13 травня 2020): 760. http://dx.doi.org/10.3390/biom10050760.

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Анотація:
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder manifesting with upper and lower neuron loss, leading to impairments in voluntary muscle function and atrophy. Mitochondrial dysfunction in metabolism and morphology have been implicated in the pathogenesis of ALS, including atypical oxidative metabolism, reduced mitochondrial respiration in muscle, and protein aggregates in the mitochondrial outer membrane. Peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α) plays an essential role in the regulation of mitochondrial biogenesis, the process by which existing mitochondria grow and divide. PGC-1α has been previously reported to be downregulated in the spinal cord of individuals with ALS. Towards targeting PGC-1α as a therapeutic mechanism, we have previously reported improved motor function and survival in the SOD1G93A mouse model of ALS by neuron-specific over-expression of PGC-1α under a neuron-specific enolase (NSE) promoter. As pharmacological intervention targeting PGC-1α would result in whole-body upregulation of this transcriptional co-activator, in the current study we investigated whether global expression of PGC-1α is beneficial in a SOD1G93A mouse model, by generating transgenic mice with PGC-1α transgene expression driven by an actin promoter. Actin-PGC-1α expression levels were assayed and confirmed in spinal cord, brain, muscle, liver, kidney, and spleen. To determine the therapeutic effects of global expression of PGC-1α, wild-type, actin-PGC-1α, SOD1G93A, and actin-PGC-1α/SOD1G93A animals were monitored for weight loss, motor performance by accelerating rotarod test, and survival. Overexpression of actin-PGC-1α did not confer significant improvement in these assessed outcomes. A potential explanation for this difference is that the actin promoter may not induce levels of PGC-1α relevant to disease pathophysiology in the cells that are specifically relevant to the pathogenesis of ALS. This evidence strongly supports future therapeutic approaches that target PGC-1α primarily in neurons.
18

Koschnitzky, J. E., K. A. Quinlan, T. J. Lukas, E. Kajtaz, E. J. Kocevar, W. F. Mayers, T. Siddique, and C. J. Heckman. "Effect of fluoxetine on disease progression in a mouse model of ALS." Journal of Neurophysiology 111, no. 11 (June 1, 2014): 2164–76. http://dx.doi.org/10.1152/jn.00425.2013.

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Selective serotonin reuptake inhibitors (SSRIs) and other antidepressants are often prescribed to amyotrophic lateral sclerosis (ALS) patients; however, the impact of these prescriptions on ALS disease progression has not been systematically tested. To determine whether SSRIs impact disease progression, fluoxetine (Prozac, 5 or 10 mg/kg) was administered to mutant superoxide dismutase 1 (SOD1) mice during one of three age ranges: neonatal [postnatal day (P)5–11], adult presymptomatic (P30 to end stage), and adult symptomatic (P70 to end stage). Long-term adult fluoxetine treatment (started at either P30 or P70 and continuing until end stage) had no significant effect on disease progression. In contrast, neonatal fluoxetine treatment (P5-11) had two effects. First, all animals (mutant SOD1G93A and control: nontransgenic and SOD1WT) receiving the highest dose (10 mg/kg) had a sustained decrease in weight from P30 onward. Second, the high-dose SOD1G93A mice reached end stage ∼8 days (∼6% decrease in life span) sooner than vehicle and low-dose animals because of an increased rate of motor impairment. Fluoxetine increases synaptic serotonin (5-HT) levels, which is known to increase spinal motoneuron excitability. We confirmed that 5-HT increases spinal motoneuron excitability during this neonatal time period and therefore hypothesized that antagonizing 5-HT receptors during the same time period would improve disease outcome. However, cyproheptadine (1 or 5 mg/kg), a 5-HT receptor antagonist, had no effect on disease progression. These results show that a brief period of antidepressant treatment during a critical time window (the transition from neonatal to juvenile states) can be detrimental in ALS mouse models.
19

Taes, Ines, Mieke Timmers, Nicole Hersmus, André Bento-Abreu, Ludo Van Den Bosch, Philip Van Damme, Johan Auwerx, and Wim Robberecht. "Hdac6 deletion delays disease progression in the SOD1G93A mouse model of ALS." Human Molecular Genetics 22, no. 9 (January 30, 2013): 1783–90. http://dx.doi.org/10.1093/hmg/ddt028.

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20

Dibaj, Payam, Eike D. Schomburg, and Heinz Steffens. "Contractile characteristics of gastrocnemius-soleus muscle in the SOD1G93A ALS mouse model." Neurological Research 37, no. 8 (April 28, 2015): 693–702. http://dx.doi.org/10.1179/1743132815y.0000000039.

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21

Zeldich, Ella, Ci-Di Chen, Emma Boden, Bryce Howat, Jason S. Nasse, Dean Zeldich, Anthony G. Lambert, et al. "Klotho Is Neuroprotective in the Superoxide Dismutase (SOD1G93A) Mouse Model of ALS." Journal of Molecular Neuroscience 69, no. 2 (June 27, 2019): 264–85. http://dx.doi.org/10.1007/s12031-019-01356-2.

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22

Schuster, J. E., R. Fu, T. Siddique, and C. J. Heckman. "Effect of prolonged riluzole exposure on cultured motoneurons in a mouse model of ALS." Journal of Neurophysiology 107, no. 1 (January 2012): 484–92. http://dx.doi.org/10.1152/jn.00714.2011.

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Riluzole is the only FDA-approved drug to treat amyotrophic lateral sclerosis, but its long-term effects on motoneurons are unknown. Therefore, we treated primary mouse spinal cord cultures with 2 μM riluzole for 4–9 days and then used whole cell patch clamp to record the passive and active properties of both wild-type and SOD1G93A motoneurons. At this concentration, riluzole blocks >50% of the sodium component of a persistent inward current that plays a major role in determining motoneuron excitability. Prolonged riluzole treatment significantly decreased the amplitude of the persistent inward current. This effect was specific for SOD1G93A motoneurons, where the amplitude decreased by 55.4%. In addition, prolonged treatment hyperpolarized the resting membrane potential as well as the voltage onset and voltage maximum of the persistent inward current (∼2–3 mV in each case). These effects appeared to offset one another and resulted in no change in the firing properties. In a subset of cells, acute reapplication of 2 μM riluzole during the recording decreased repetitive firing and the persistent inward current, which is consistent with the normal effects of riluzole. The downregulation of the persistent inward current in response to prolonged riluzole administration is in contrast to the strong upregulation of this same current after descending neuromodulatory drive to the cord is lost following spinal injury. This dichotomy suggests that decreased activation of G protein-coupled pathways can induce upregulation in the persistent inward current but that direct channel block is ineffective.
23

Tan, Honglin, Mina Chen, Dejiang Pang, Xiaoqiang Xia, Chongyangzi Du, Wanchun Yang, Yiyuan Cui, et al. "LanCL1 promotes motor neuron survival and extends the lifespan of amyotrophic lateral sclerosis mice." Cell Death & Differentiation 27, no. 4 (September 30, 2019): 1369–82. http://dx.doi.org/10.1038/s41418-019-0422-6.

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Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of motor neurons. Improving neuronal survival in ALS remains a significant challenge. Previously, we identified Lanthionine synthetase C-like protein 1 (LanCL1) as a neuronal antioxidant defense gene, the genetic deletion of which causes apoptotic neurodegeneration in the brain. Here, we report in vivo data using the transgenic SOD1G93A mouse model of ALS indicating that CNS-specific expression of LanCL1 transgene extends lifespan, delays disease onset, decelerates symptomatic progression, and improves motor performance of SOD1G93A mice. Conversely, CNS-specific deletion of LanCL1 leads to neurodegenerative phenotypes, including motor neuron loss, neuroinflammation, and oxidative damage. Analysis reveals that LanCL1 is a positive regulator of AKT activity, and LanCL1 overexpression restores the impaired AKT activity in ALS model mice. These findings indicate that LanCL1 regulates neuronal survival through an alternative mechanism, and suggest a new therapeutic target in ALS.
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Méndez-López, Iago, Francisco J. Sancho-Bielsa, Tobias Engel, Antonio G. G. García, and Juan Fernando Padín. "Progressive Mitochondrial SOD1G93A Accumulation Causes Severe Structural, Metabolic and Functional Aberrations through OPA1 Down-Regulation in a Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 22, no. 15 (July 30, 2021): 8194. http://dx.doi.org/10.3390/ijms22158194.

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In recent years, the “non-autonomous motor neuron death” hypothesis has become more consolidated behind amyotrophic lateral sclerosis (ALS). It postulates that cells other than motor neurons participate in the pathology. In fact, the involvement of the autonomic nervous system is fundamental since patients die of sudden death when they become unable to compensate for cardiorespiratory arrest. Mitochondria are thought to play a fundamental role in the physiopathology of ALS, as they are compromised in multiple ALS models in different cell types, and it also occurs in other neurodegenerative diseases. Our study aimed to uncover mitochondrial alterations in the sympathoadrenal system of a mouse model of ALS, from a structural, bioenergetic and functional perspective during disease instauration. We studied the adrenal chromaffin cell from mutant SOD1G93A mouse at pre-symptomatic and symptomatic stages. The mitochondrial accumulation of the mutated SOD1G93A protein and the down-regulation of optic atrophy protein-1 (OPA1) provoke mitochondrial ultrastructure alterations prior to the onset of clinical symptoms. These changes affect mitochondrial fusion dynamics, triggering mitochondrial maturation impairment and cristae swelling, with increased size of cristae junctions. The functional consequences are a loss of mitochondrial membrane potential and changes in the bioenergetics profile, with reduced maximal respiration and spare respiratory capacity of mitochondria, as well as enhanced production of reactive oxygen species. This study identifies mitochondrial dynamics regulator OPA1 as an interesting therapeutic target in ALS. Additionally, our findings in the adrenal medulla gland from presymptomatic stages highlight the relevance of sympathetic impairment in this disease. Specifically, we show new SOD1G93A toxicity pathways affecting cellular energy metabolism in non-motor neurons, which offer a possible link between cell specific metabolic phenotype and the progression of ALS.
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Valdebenito-Maturana, Braulio, Esteban Arancibia, Gonzalo Riadi, Juan Carlos Tapia, and Mónica Carrasco. "Locus-specific analysis of Transposable Elements during the progression of ALS in the SOD1G93A mouse model." PLOS ONE 16, no. 10 (October 6, 2021): e0258291. http://dx.doi.org/10.1371/journal.pone.0258291.

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Transposable Elements (TEs) are ubiquitous genetic elements with the ability to move within a genome. TEs contribute to a large fraction of the repetitive elements of a genome, and because of their nature, they are not routinely analyzed in RNA-Seq gene expression studies. Amyotrophic Lateral Sclerosis (ALS) is a lethal neurodegenerative disease, and a well-accepted model for its study is the mouse harboring the human SOD1G93A mutant. In this model, landmark stages of the disease can be recapitulated at specific time points, making possible to understand changes in gene expression across time. While there are several works reporting TE activity in ALS models, they have not explored their activity through the disease progression. Moreover, they have done it at the expense of losing their locus of expression. Depending on their genomic location, TEs can regulate genes in cis and in trans, making locus-specific analysis of TEs of importance in order to understand their role in modulating gene expression. Particularly, the locus-specific role of TEs in ALS has not been fully elucidated. In this work, we analyzed publicly available RNA-Seq datasets of the SOD1G93A mouse model, to understand the locus-specific role of TEs. We show that TEs become up-regulated at the early stages of the disease, and via statistical associations, we speculate that they can regulate several genes, which in turn might be contributing to the genetic dysfunction observed in ALS.
26

Ogbu, Destiny, Yongguo Zhang, Katerina Claud, Yinglin Xia, and Jun Sun. "Target Metabolites to Slow Down Progression of Amyotrophic Lateral Sclerosis in Mice." Metabolites 12, no. 12 (December 12, 2022): 1253. http://dx.doi.org/10.3390/metabo12121253.

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Microbial metabolites affect the neuron system and muscle cell functions. Amyotrophic lateral sclerosis (ALS) is a multifactorial neuromuscular disease. Our previous study has demonstrated elevated intestinal inflammation and dysfunction of the microbiome in patients with ALS and an ALS mouse model (human-SOD1G93A transgenic mice). However, the metabolites in ALS progression are unknown. Using an unbiased global metabolomic measurement and targeted measurement, we investigated the longitudinal changes of fecal metabolites in SOD1G93A mice over the course of 13 weeks. We further compared the changes of metabolites and inflammatory response in age-matched wild-type (WT) and SOD1G93A mice treated with the bacterial product butyrate. We found changes in carbohydrate levels, amino acid metabolism, and the formation of gamma-glutamyl amino acids. Shifts in several microbially contributed catabolites of aromatic amino acids agree with butyrate-induced changes in the composition of the gut microbiome. Declines in gamma-glutamyl amino acids in feces may stem from differential expression of gamma-glutamyltransferase (GGT) in response to butyrate administration. Due to the signaling nature of amino acid-derived metabolites, these changes indicate changes in inflammation, e.g., histamine, and contribute to differences in systemic levels of neurotransmitters, e.g., γ-Aminobutyric acid (GABA) and glutamate. Butyrate treatment was able to restore some of the healthy metabolites in ALS mice. Moreover, microglia in the spinal cord were measured by IBA1 staining. Butyrate treatment significantly suppressed the IBA1 level in the SOD1G93A mice. Serum IL-17 and LPS were significantly reduced in the butyrate-treated SOD1G93A mice. We have demonstrated an inter-organ communications link among microbial metabolites, neuroactive metabolites from the gut, and inflammation in ALS progression. The study supports the potential to use metabolites as ALS hallmarks and for treatment.
27

Pambo-Pambo, Arnaud, Jacques Durand, and Jean-Patrick Gueritaud. "Early Excitability Changes in Lumbar Motoneurons of Transgenic SOD1G85R and SOD1G93A-Low Mice." Journal of Neurophysiology 102, no. 6 (December 2009): 3627–42. http://dx.doi.org/10.1152/jn.00482.2009.

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This work characterizes the properties of wild-type (WT) mouse motoneurons in the second postnatal week and compares these at the same age and in the same conditions to those of two different SOD1 mutant lines used as models of human amyotrophic lateral sclerosis (ALS), the SOD1G93A low expressor line and SOD1G85R line, to describe any changes in the functional properties of mutant motoneurons (Mns) that may be related to the pathogenesis of human ALS. We show that very early changes in excitability occur in SOD1 mutant Mns that have different properties from those of WT animals. The SOD1G93A-Low low expressor line displays specific differences that are not found in other mutant lines including a more depolarized membrane potential, larger spike width, and slower spike rise slope. With current pulses SOD1G93A-Low were hyperexcitable, but both mutants had a lower gain with current ramps stimulation. Changes in the threshold and intensities of Na+ and Ca2+ persistent inward currents were also observed. Low expressor mutants show reduced total persistant inward currents compared with WT motoneurons in the same recording conditions and give arguments toward modifications of the balance between Na+ and Ca2+ persistent inward currents. During the second week postnatal, SOD1G93A-Low lumbar motoneurons appear more immature than those of SOD1G85R compared with WT and we propose that different time course of the disease, possibly linked with different toxic properties of the mutated protein in each model, may explain the discrepancies between excitability changes described in the different models.
28

Spalloni, Alida, Viviana Greco, Giulia Ciriminna, Victor Corasolla Carregari, Federica Marini, Luisa Pieroni, Nicola B. Mercuri, Andrea Urbani, and Patrizia Longone. "Impact of Pharmacological Inhibition of Hydrogen Sulphide Production in the SOD1G93A-ALS Mouse Model." International Journal of Molecular Sciences 20, no. 10 (May 24, 2019): 2550. http://dx.doi.org/10.3390/ijms20102550.

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A number of factors can trigger amyotrophic lateral sclerosis (ALS), although its precise pathogenesis is still uncertain. In a previous study done by us, poisonous liquoral levels of hydrogen sulphide (H2S) in sporadic ALS patients were reported. In the same study very high concentrations of H2S in the cerebral tissues of the familial ALS (fALS) model of the SOD1G93A mouse, were measured. The objective of this study was to test whether decreasing the levels of H2S in the fALS mouse could be beneficial. Amino-oxyacetic acid (AOA)—a systemic dual inhibitor of cystathionine-β-synthase and cystathionine-γ lyase (two key enzymes in the production of H2S)—was administered to fALS mice. AOA treatment decreased the content of H2S in the cerebral tissues, and the lifespan of female mice increased by approximately ten days, while disease progression in male mice was not affected. The histological evaluation of the spinal cord of the females revealed a significant increase in GFAP positivity and a significant decrease in IBA1 positivity. In conclusion, the results of the study indicate that, in the animal model, the inhibition of H2S production is more effective in females. The findings reinforce the need to adequately consider sex as a relevant factor in ALS.
29

OLIVÁN, Sara, Ana Cristina CALVO, Amaya RANDO, María Jesús MUÑOZ, Pilar ZARAGOZA, and Rosario OSTA. "Comparative study of behavioural tests in the SOD1G93A mouse model of amyotrophic lateral sclerosis." Experimental Animals 64, no. 2 (2015): 147–53. http://dx.doi.org/10.1538/expanim.14-0077.

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30

Dutta, Kallol, Priyanka Patel, and Jean-Pierre Julien. "Protective effects of Withania somnifera extract in SOD1G93A mouse model of amyotrophic lateral sclerosis." Experimental Neurology 309 (November 2018): 193–204. http://dx.doi.org/10.1016/j.expneurol.2018.08.008.

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31

Schomburg, Eike D., Heinz Steffens, Jana Zschüntzsch, Payam Dibaj, and Bernhard U. Keller. "Fatigability of spinal reflex transmission in a mouse model (SOD1G93A ) of amyotrophic lateral sclerosis." Muscle & Nerve 43, no. 2 (January 19, 2011): 230–36. http://dx.doi.org/10.1002/mus.21835.

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32

Clark, Courtney M., Rosemary M. Clark, Joshua A. Hoyle, Jyoti A. Chuckowree, Catriona A. McLean, and Tracey C. Dickson. "Differential NPY-Y1 Receptor Density in the Motor Cortex of ALS Patients and Familial Model of ALS." Brain Sciences 11, no. 8 (July 23, 2021): 969. http://dx.doi.org/10.3390/brainsci11080969.

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Destabilization of faciliatory and inhibitory circuits is an important feature of corticomotor pathology in amyotrophic lateral sclerosis (ALS). While GABAergic inputs to upper motor neurons are reduced in models of the disease, less understood is the involvement of peptidergic inputs to upper motor neurons in ALS. The neuropeptide Y (NPY) system has been shown to confer neuroprotection against numerous pathogenic mechanisms implicated in ALS. However, little is known about how the NPY system functions in the motor system. Herein, we investigate post-synaptic NPY signaling on upper motor neurons in the rodent and human motor cortex, and on cortical neuron populations in vitro. Using immunohistochemistry, we show the increased density of NPY-Y1 receptors on the soma of SMI32-positive upper motor neurons in post-mortem ALS cases and SOD1G93A excitatory cortical neurons in vitro. Analysis of receptor density on Thy1-YFP-H-positive upper motor neurons in wild-type and SOD1G93A mouse tissue revealed that the distribution of NPY-Y1 receptors was changed on the apical processes at early-symptomatic and late-symptomatic disease stages. Together, our data demonstrate the differential density of NPY-Y1 receptors on upper motor neurons in a familial model of ALS and in ALS cases, indicating a novel pathway that may be targeted to modulate upper motor neuron activity.
33

Pascali, Giancarlo, Daniele Panetta, Mariarosaria De Simone, Silvia Burchielli, Valentina Lucchesi, Elena Sanguinetti, Simone Zanoni, et al. "Preliminary Investigation of a Novel 18F Radiopharmaceutical for Imaging CB2 Receptors in a SOD Mouse Model." Australian Journal of Chemistry 74, no. 6 (2021): 443. http://dx.doi.org/10.1071/ch20247.

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We successfully radiolabelled a novel prospective cannabinoid type 2 receptor ligand with 18F and tested its biodistribution in animal models by positron emission tomography (PET)/computed tomography (CT) imaging. The radiolabelling process was conducted on an alkyl mesylate fragment of the main naphthyridine core, using highly efficient microfluidic technology. No preliminary protection was needed, and the product was purified by semi-prep HPLC and SPE formulation, allowing the desired diastereomeric mixture to be obtained in 29% radiochemical yield and&gt;95% radiochemically pure. SOD1G93A mice were used as model of overexpression of CB2 receptors; PET imaging revealed a significant increase of the tracer distribution volume in the brain of symptomatic subjects compared with the asymptomatic ones.
34

Violatto, Martina Bruna, Laura Pasetto, Elisabetta Casarin, Camilla Tondello, Elisa Schiavon, Laura Talamini, Gloria Marchini, et al. "Development of a Nanoparticle-Based Approach for the Blood–Brain Barrier Passage in a Murine Model of Amyotrophic Lateral Sclerosis." Cells 11, no. 24 (December 10, 2022): 4003. http://dx.doi.org/10.3390/cells11244003.

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The development of nanoparticles (NPs) to enable the passage of drugs across blood–brain barrier (BBB) represents one of the main challenges in neuropharmacology. In recent years, NPs that are able to transport drugs and interact with brain endothelial cells have been tested. Here, we investigated whether the functionalization of avidin-nucleic-acid-nanoassembly (ANANAS) with apolipoprotein E (ApoE) would allow BBB passage in the SOD1G93A mouse model of amyotrophic lateral sclerosis. Our results demonstrated that ANANAS was able to transiently cross BBB to reach the central nervous system (CNS), and ApoE did not enhance this property. Next, we investigated if ANANAS could improve CNS drug delivery. To this aim, the steroid dexamethasone was covalently linked to ANANAS through an acid-reversible hydrazone bond. Our data showed that the steroid levels in CNS tissues of SOD1G93A mice treated with nanoformulation were below the detection limit. This result demonstrates that the passage of BBB is not sufficient to guarantee the release of the cargo in CNS and that a different strategy for drug tethering should be devised. The present study furthermore highlights that NPs can be useful in improving the passage through biological barriers but may limit the interaction of the therapeutic compound with the specific target.
35

Novoselov, Sergey S., Wendy J. Mustill, Anna L. Gray, James R. Dick, Naheed Kanuga, Bernadett Kalmar, Linda Greensmith, and Michael E. Cheetham. "Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis." PLoS ONE 8, no. 8 (August 30, 2013): e73944. http://dx.doi.org/10.1371/journal.pone.0073944.

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36

Seki, Soju, Toru Yamamoto, Kiara Quinn, Igor Spigelman, Antonios Pantazis, Riccardo Olcese, Martina Wiedau-Pazos, Scott H. Chandler, and Sharmila Venugopal. "Circuit-Specific Early Impairment of Proprioceptive Sensory Neurons in the SOD1G93A Mouse Model for ALS." Journal of Neuroscience 39, no. 44 (September 17, 2019): 8798–815. http://dx.doi.org/10.1523/jneurosci.1214-19.2019.

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37

Boërio, Delphine, Bernadett Kalmar, Linda Greensmith, and Hugh Bostock. "Excitability properties of mouse motor axons in the mutant SOD1G93A model of amyotrophic lateral sclerosis." Muscle & Nerve 41, no. 6 (January 21, 2010): 774–84. http://dx.doi.org/10.1002/mus.21579.

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38

Fabbrizio, Paola, Savina Apolloni, Andrea Bianchi, Illari Salvatori, Cristiana Valle, Chiara Lanzuolo, Caterina Bendotti, Giovanni Nardo, and Cinzia Volonté. "P2X7 activation enhances skeletal muscle metabolism and regeneration in SOD1G93A mouse model of amyotrophic lateral sclerosis." Brain Pathology 30, no. 2 (August 18, 2019): 272–82. http://dx.doi.org/10.1111/bpa.12774.

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39

Bryson, J. Barney, Carl Hobbs, Michael J. Parsons, Karen D. Bosch, Amelie Pandraud, Frank S. Walsh, Patrick Doherty, and Linda Greensmith. "Amyloid precursor protein (APP) contributes to pathology in the SOD1G93A mouse model of amyotrophic lateral sclerosis." Human Molecular Genetics 21, no. 17 (June 7, 2012): 3871–82. http://dx.doi.org/10.1093/hmg/dds215.

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40

Lepore, Angelo C., John O'Donnell, Andrew S. Kim, Timothy Williams, Alicia Tuteja, Mahendra S. Rao, Linda L. Kelley, James T. Campanelli, and Nicholas J. Maragakis. "Human Glial-Restricted Progenitor Transplantation into Cervical Spinal Cord of the SOD1G93A Mouse Model of ALS." PLoS ONE 6, no. 10 (October 5, 2011): e25968. http://dx.doi.org/10.1371/journal.pone.0025968.

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41

Hegedus, J., C. T. Putman, and T. Gordon. "Time course of preferential motor unit loss in the SOD1G93A mouse model of amyotrophic lateral sclerosis." Neurobiology of Disease 28, no. 2 (November 2007): 154–64. http://dx.doi.org/10.1016/j.nbd.2007.07.003.

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42

Sábado, J., A. Casanovas, H. Rodrigo, G. Arqué, and J. E. Esquerda. "Adverse effects of a SOD1-peptide immunotherapy on SOD1G93A mouse slow model of amyotrophic lateral sclerosis." Neuroscience 310 (December 2015): 38–50. http://dx.doi.org/10.1016/j.neuroscience.2015.09.027.

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43

Bianchi, F., C. Rossi, L. Muzio, N. Riva, C. Butera, M. Cursi, S. Amadio, G. Comi, A. Quattrini, and U. Del Carro. "54. Early neurophysiological signs of disease in SOD1G93A mouse model: A stimulated single-fiber electromyography study." Clinical Neurophysiology 127, no. 12 (December 2016): e336. http://dx.doi.org/10.1016/j.clinph.2016.10.066.

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44

Kang, Jihong, and Serge Rivest. "MyD88-deficient bone marrow cells accelerate onset and reduce survival in a mouse model of amyotrophic lateral sclerosis." Journal of Cell Biology 179, no. 6 (December 17, 2007): 1219–30. http://dx.doi.org/10.1083/jcb.200705046.

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Increasing evidence suggests that neurotoxicity of secreted superoxide dismutase 1 (SOD1) mutants is associated with amyotrophic lateral sclerosis (ALS). We show here that mutant SOD1 protein activates microglia via a myeloid differentiation factor 88 (MyD88)–dependent pathway. This inflammatory response is also associated with a marked recruitment of bone marrow–derived microglia (BMDM) in the central nervous system. We then generated chimeric SOD1G37R and SOD1G93A mice by transplantation of bone marrow (BM) cells from MyD88-deficient or green fluorescent protein (GFP)–expressing mice. SOD1G37R mice receiving MyD88−/− BM cells exhibit a significantly earlier disease onset and shorter lifespan compared with mice transplanted with control GFP cells. This compelling beneficial effect of MyD88-competent BMDM is a previously unrecognized natural innate immune mechanism of neuroprotection in a mouse model of late-onset motor neuron disease.
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Vallarola, Antonio, Massimo Tortarolo, Roberta De Gioia, Luisa Iamele, Hugo de Jonge, Giovanni de Nola, Enrica Bovio, et al. "A Novel HGF/SF Receptor (MET) Agonist Transiently Delays the Disease Progression in an Amyotrophic Lateral Sclerosis Mouse Model by Promoting Neuronal Survival and Dampening the Immune Dysregulation." International Journal of Molecular Sciences 21, no. 22 (November 12, 2020): 8542. http://dx.doi.org/10.3390/ijms21228542.

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Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease with no effective treatment. The Hepatocyte Growth Factor/Scatter Factor (HGF/SF), through its receptor MET, is one of the most potent survival-promoting factors for motor neurons (MN) and is known as a modulator of immune cell function. We recently developed a novel recombinant MET agonist optimized for therapy, designated K1K1. K1K1 was ten times more potent than HGF/SF in preventing MN loss in an in vitro model of ALS. Treatments with K1K1 delayed the onset of muscular impairment and reduced MN loss and skeletal muscle denervation of superoxide dismutase 1 G93A (SOD1G93A) mice. This effect was associated with increased levels of phospho-extracellular signal-related kinase (pERK) in the spinal cord and sciatic nerves and the activation of non-myelinating Schwann cells. Moreover, reduced activated microglia and astroglia, lower T cells infiltration and increased interleukin 4 (IL4) levels were found in the lumbar spinal cord of K1K1 treated mice. K1K1 treatment also prevented the infiltration of T cells in skeletal muscle of SOD1G93A mice. All these protective effects were lost on long-term treatment suggesting a mechanism of drug tolerance. These data provide a rational justification for further exploring the long-term loss of K1K1 efficacy in the perspective of providing a potential treatment for ALS.
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Flis, Damian Jozef, Katarzyna Dzik, Jan Jacek Kaczor, Karol Cieminski, Malgorzata Halon-Golabek, Jedrzej Antosiewicz, Mariusz Roman Wieckowski, and Wieslaw Ziolkowski. "Swim Training Modulates Mouse Skeletal Muscle Energy Metabolism and Ameliorates Reduction in Grip Strength in a Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 20, no. 2 (January 9, 2019): 233. http://dx.doi.org/10.3390/ijms20020233.

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Metabolic reprogramming in skeletal muscles in the human and animal models of amyotrophic lateral sclerosis (ALS) may be an important factor in the diseases progression. We hypothesized that swim training, a modulator of cellular metabolism via changes in muscle bioenergetics and oxidative stress, ameliorates the reduction in muscle strength in ALS mice. In this study, we used transgenic male mice with the G93A human SOD1 mutation B6SJL-Tg (SOD1G93A) 1Gur/J and wild type B6SJL (WT) mice. Mice were subjected to a grip strength test and isolated skeletal muscle mitochondria were used to perform high-resolution respirometry. Moreover, the activities of enzymes involved in the oxidative energy metabolism and total sulfhydryl groups (as an oxidative stress marker) were evaluated in skeletal muscle. ALS reduces muscle strength (−70% between 11 and 15 weeks, p < 0.05), modulates muscle metabolism through lowering citrate synthase (CS) (−30% vs. WT, p = 0.0007) and increasing cytochrome c oxidase and malate dehydrogenase activities, and elevates oxidative stress markers in skeletal muscle. Swim training slows the reduction in muscle strength (−5% between 11 and 15 weeks) and increases CS activity (+26% vs. ALS I, p = 0.0048). Our findings indicate that swim training is a modulator of skeletal muscle energy metabolism with concomitant improvement of skeletal muscle function in ALS mice.
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Mead, Richard J., Ellen J. Bennett, Aneurin J. Kennerley, Paul Sharp, Claire Sunyach, Paul Kasher, Jason Berwick, et al. "Optimised and Rapid Pre-clinical Screening in the SOD1G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis (ALS)." PLoS ONE 6, no. 8 (August 18, 2011): e23244. http://dx.doi.org/10.1371/journal.pone.0023244.

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48

Huang, G., X. Lee, Y. Bian, Z. Shao, G. Sheng, R. B. Pepinsky, and S. Mi. "Death receptor 6 (DR6) antagonist antibody is neuroprotective in the mouse SOD1G93A model of amyotrophic lateral sclerosis." Cell Death & Disease 4, no. 10 (October 2013): e841-e841. http://dx.doi.org/10.1038/cddis.2013.378.

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49

Noh, Min Young, Kyung Ah Cho, Heejaung Kim, Sung-Min Kim, and Seung Hyun Kim. "Erythropoietin modulates the immune-inflammatory response of a SOD1G93A transgenic mouse model of amyotrophic lateral sclerosis (ALS)." Neuroscience Letters 574 (June 2014): 53–58. http://dx.doi.org/10.1016/j.neulet.2014.05.001.

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50

Rei, N., D. M. Rombo, M. F. Ferreira, Y. Baqi, C. E. Müller, J. A. Ribeiro, A. M. Sebastião, and S. H. Vaz. "Hippocampal synaptic dysfunction in the SOD1G93A mouse model of Amyotrophic Lateral Sclerosis: Reversal by adenosine A2AR blockade." Neuropharmacology 171 (July 2020): 108106. http://dx.doi.org/10.1016/j.neuropharm.2020.108106.

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