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Journal articles on the topic 'VLFCA'

Author: Grafiati

Published: 12 October 2024

Last updated: 31 July 2025

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1

Zhou, Jie, Marcia R. Terluk, Paul J. Orchard, James C. Cloyd, and Reena V. Kartha. "N-Acetylcysteine Reverses the Mitochondrial Dysfunction Induced by Very Long-Chain Fatty Acids in Murine Oligodendrocyte Model of Adrenoleukodystrophy." Biomedicines 9, no. 12 (2021): 1826. http://dx.doi.org/10.3390/biomedicines9121826.

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The accumulation of saturated very long-chain fatty acids (VLCFA, ≥C22:0) due to peroxisomal impairment leads to oxidative stress and neurodegeneration in X-linked adrenoleukodystrophy (ALD). Among the neural supporting cells, myelin-producing oligodendrocytes are the most sensitive to the detrimental effect of VLCFA. Here, we characterized the mitochondrial dysfunction and cell death induced by VLFCA, and examined whether N-acetylcysteine (NAC), an antioxidant, prevents the cytotoxicity. We exposed murine oligodendrocytes (158 N) to hexacosanoic acid (C26:0, 1–100 µM) for 24 h and measured re

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McGuinness, M. C., J. F. Lu, H. P. Zhang, et al. "Role of ALDP (ABCD1) and Mitochondria in X-Linked Adrenoleukodystrophy." Molecular and Cellular Biology 23, no. 2 (2003): 744–53. http://dx.doi.org/10.1128/mcb.23.2.744-753.2003.

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ABSTRACT Peroxisomal disorders have been associated with malfunction of peroxisomal metabolic pathways, but the pathogenesis of these disorders is largely unknown. X-linked adrenoleukodystrophy (X-ALD) is associated with elevated levels of very-long-chain fatty acids (VLCFA; C>22:0) that have been attributed to reduced peroxisomal VLCFA β-oxidation activity. Previously, our laboratory and others have reported elevated VLCFA levels and reduced peroxisomal VLCFA β-oxidation in human and mouse X-ALD fibroblasts. In this study, we found normal levels of peroxisomal VLCFA β-oxidation in tissues

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Siigur, Jüri, Katrin Trummal, Külli TÕnismägi, et al. "Use of MALDI-TOF Mass Spectrometry for Specificity Studies of Biomedically Important Proteases." Spectroscopy 16, no. 3-4 (2002): 161–70. http://dx.doi.org/10.1155/2002/204307.

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Proteases play crucial role starting from fertilization until to cell death. Our studies of the two Viperidae venoms (Levantine viperVipera lebetina, Common viperVipera berus) have demonstrated the existence of biomedically important proteases, both coagulants and anticoagulants that may be useful as diagnostic tools or potential therapeutics. We showed that venoms of both snakes contain: (i) metalloproteases and serine proteases that degrade fibrinogen, but not fibrin; (ii) factor X activators (VLFXA, VBFXAE); (iii) bradykinin-releasing serine proteases. AdditionallyVipera lebetinasnake venom

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Batsale, Marguerite, Delphine Bahammou, Laetitia Fouillen, Sébastien Mongrand, Jérôme Joubès, and Frédéric Domergue. "Biosynthesis and Functions of Very-Long-Chain Fatty Acids in the Responses of Plants to Abiotic and Biotic Stresses." Cells 10, no. 6 (2021): 1284. http://dx.doi.org/10.3390/cells10061284.

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Very-long-chain fatty acids (i.e., fatty acids with more than 18 carbon atoms; VLCFA) are important molecules that play crucial physiological and structural roles in plants. VLCFA are specifically present in several membrane lipids and essential for membrane homeostasis. Their specific accumulation in the sphingolipids of the plasma membrane outer leaflet is of primordial importance for its correct functioning in intercellular communication. VLCFA are found in phospholipids, notably in phosphatidylserine and phosphatidylethanolamine, where they could play a role in membrane domain organization

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Zhukov, Anatoly, and Valery Popov. "Synthesis of C20–38 Fatty Acids in Plant Tissues." International Journal of Molecular Sciences 23, no. 9 (2022): 4731. http://dx.doi.org/10.3390/ijms23094731.

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Very-long-chain fatty acids (VLCFA) are involved in a number of important plant physiological functions. Disorders in the expression of genes involved in the synthesis of VLCFA lead to a number of phenotypic consequences, ranging from growth retardation to the death of embryos. The elongation of VLCFA in the endoplasmic reticulum (ER) is carried out by multiple elongase complexes with different substrate specificities and adapted to the synthesis of a number of products required for a number of metabolic pathways. The information about the enzymes involved in the synthesis of VLCFA with more t

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Zhukov, Anatoly, and Valery Popov. "Synthesis of C20–38 Fatty Acids in Plant Tissues." International Journal of Molecular Sciences 23, no. 9 (2022): 4731. http://dx.doi.org/10.3390/ijms23094731.

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Very-long-chain fatty acids (VLCFA) are involved in a number of important plant physiological functions. Disorders in the expression of genes involved in the synthesis of VLCFA lead to a number of phenotypic consequences, ranging from growth retardation to the death of embryos. The elongation of VLCFA in the endoplasmic reticulum (ER) is carried out by multiple elongase complexes with different substrate specificities and adapted to the synthesis of a number of products required for a number of metabolic pathways. The information about the enzymes involved in the synthesis of VLCFA with more t

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Bourassa, Dianna V., Elmar L. Kannenberg, D. Janine Sherrier, R. Jeffrey Buhr, and Russell W. Carlson. "The Lipopolysaccharide Lipid A Long-Chain Fatty Acid Is Important for Rhizobium leguminosarum Growth and Stress Adaptation in Free-Living and Nodule Environments." Molecular Plant-Microbe Interactions® 30, no. 2 (2017): 161–75. http://dx.doi.org/10.1094/mpmi-11-16-0230-r.

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Rhizobium bacteria live in soil and plant environments, are capable of inducing symbiotic nodules on legumes, invade these nodules, and develop into bacteroids that fix atmospheric nitrogen into ammonia. Rhizobial lipopolysaccharide (LPS) is anchored in the bacterial outer membrane through a specialized lipid A containing a very long-chain fatty acid (VLCFA). VLCFA function for rhizobial growth in soil and plant environments is not well understood. Two genes, acpXL and lpxXL, encoding acyl carrier protein and acyltransferase, are among the six genes required for biosynthesis and transfer of VL

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Robinson, B. S., D. W. Johnson, and A. Poulos. "Unique molecular species of phosphatidylcholine containing very-long-chain (C24-C38) polyenoic fatty acids in rat brain." Biochemical Journal 265, no. 3 (1990): 763–67. http://dx.doi.org/10.1042/bj2650763.

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Rat brain has been shown to contain polyenoic very-long-chain fatty acids (VLCFA) belonging to the n-3 and n-6 series with four, five and six double bonds and even-carbon chain lengths from 24 to 38. These fatty acids are almost exclusively located in unusual molecular species of phosphatidylcholine at the sn-1 position of the glycerol backbone, whereas saturated, monoenoic and polyenoic fatty acids with less than 24 carbon atoms are present at the sn-2 position. Polyenoic VLCFA phosphatidylcholine in neonatal rat brain is enriched with n-6 pentaenoic and n-3 hexaenoic VLCFA with up to 36 carb

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Trinh, Duy-Chi, Julien Lavenus, Tatsuaki Goh, et al. "PUCHI regulates very long chain fatty acid biosynthesis during lateral root and callus formation." Proceedings of the National Academy of Sciences 116, no. 28 (2019): 14325–30. http://dx.doi.org/10.1073/pnas.1906300116.

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Lateral root organogenesis plays an essential role in elaborating plant root system architecture. InArabidopsis, the AP2 family transcription factor PUCHI controls cell proliferation in lateral root primordia. To identify potential targets of PUCHI, we analyzed a time course transcriptomic dataset of lateral root formation. We report that multiple genes coding for very long chain fatty acid (VLCFA) biosynthesis enzymes are induced during lateral root development in a PUCHI-dependent manner. Significantly, several mutants perturbed in VLCFA biosynthesis show similar lateral root developmental d

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Williams, John, Kan Zhu, Eric Crampon, and André Iffland. "Fit-for-purpose biomarker LC–MS/MS qualification for the quantitation of very long chain fatty acids in human cerebrospinal fluid." Bioanalysis 12, no. 3 (2020): 143–58. http://dx.doi.org/10.4155/bio-2019-0256.

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Aim: Very long chain fatty acids (VLCFAs) have been identified as biomarkers for several peroxisomal disorders necessitating the need for reliable biomarker assays in particular C20, C22, C24, C26 in cerebrospinal fluid (CSF). Until now no absolute quantitation assay for total VLCFAs in CSF has been successfully developed and qualified for clinical use. Methodology: A quantitative LC–MS/MS assay for total VLCFA in human CSF was developed. Derivatization tag and coupling chemistry were optimized for sensitivity. CSF contamination by blood, non-specific binding of VLCFA to surfaces and exogenous

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Sharp, P., A. Poulos, A. Fellenberg, and D. Johnson. "Structure and lipid distribution of polyenoic very-long-chain fatty acids in the brain of peroxisome-deficient patients (Zellweger syndrome)." Biochemical Journal 248, no. 1 (1987): 61–67. http://dx.doi.org/10.1042/bj2480061.

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The polyenoic fatty acids with carbon chain lengths from 26 to 38 (very-long-chain fatty acids, VLCFA) previously detected in abnormal amounts in Zellweger syndrome brain have been shown to be n-6 derivatives and therefore probably derived by chain elongation of shorter-chain n-6 fatty acids such as linoleic acid and arachidonic acid. Polyenoic VLCFA are also present in Zellweger syndrome liver, but this tissue differs significantly from brain in that the saturated and mono-unsaturated derivatives are the major VLCFA. Zellweger syndrome brain polyenoic VLCFA are present in the neutral lipids p

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Jones, Eric A. L., and Micheal D. K. Owen. "Investigating the Efficacy of Selected Very-Long-Chain Fatty Acid-Inhibiting Herbicides on Iowa Waterhemp (Amaranthus tuberculatus) Populations with Evolved Multiple Herbicide Resistances." Agronomy 11, no. 3 (2021): 595. http://dx.doi.org/10.3390/agronomy11030595.

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Very long chain fatty acid (VLCFA)-inhibiting herbicides (Herbicide group (HG) 15) have been applied to corn and soybean fields in Iowa since the 1960s. The VLCFA-inhibiting herbicides are now applied more frequently to control multiple herbicide-resistant (MHR) waterhemp (Amaranthus tuberculatus Moq. J.D. Sauer) populations that are ubiquitous across the Midwest United States as resistance to the VLCFA-inhibiting herbicides is not widespread. Waterhemp has evolved multiple resistances to herbicides from seven sites of action (HG 2, 4, 5, 9, 14, 15, and 27), and six-way herbicide-resistant pop

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Micoogullari, Yagmur, Sankha S. Basu, Jessie Ang, et al. "Dysregulation of very-long-chain fatty acid metabolism causes membrane saturation and induction of the unfolded protein response." Molecular Biology of the Cell 31, no. 1 (2020): 7–17. http://dx.doi.org/10.1091/mbc.e19-07-0392.

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Increasing evidence suggests that lipid homeostasis is critical for protein quality control. Very-long-chain fatty acids (VLCFA) are rare and poorly understood species. Here, it is shown that dysregulation of VLCFA metabolism causes increased membrane saturation, endoplasmic reticulum stress, and unfolded protein response induction.

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Zhu, Xiaoyu, Frédérique Tellier, Ying Gu, and Shundai Li. "Disruption of Very-Long-Chain-Fatty Acid Synthesis Has an Impact on the Dynamics of Cellulose Synthase in Arabidopsis thaliana." Plants 9, no. 11 (2020): 1599. http://dx.doi.org/10.3390/plants9111599.

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In higher plants, cellulose is synthesized by membrane-spanning large protein complexes named cellulose synthase complexes (CSCs). In this study, the Arabidopsis PASTICCINO2 (PAS2) was identified as an interacting partner of cellulose synthases. PAS2 was previously characterized as the plant 3-hydroxy-acyl-CoA dehydratase, an ER membrane-localized dehydratase that is essential for very-long-chain-fatty acid (VLCFA) elongation. The pas2-1 mutants show defective cell elongation and reduction in cellulose content in both etiolated hypocotyls and light-grown roots. Although disruption of VLCFA syn

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van de Stadt, Stephanie I. W., Petra A. W. Mooyer, Inge M. E. Dijkstra, et al. "Biochemical Studies in Fibroblasts to Interpret Variants of Unknown Significance in the ABCD1 Gene." Genes 12, no. 12 (2021): 1930. http://dx.doi.org/10.3390/genes12121930.

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Due to newborn screening for X-linked adrenoleukodystrophy (ALD), and the use of exome sequencing in clinical practice, the detection of variants of unknown significance (VUS) in the ABCD1 gene is increasing. In these cases, functional tests in fibroblasts may help to classify a variant as (likely) benign or pathogenic. We sought to establish reference ranges for these tests in ALD patients and control subjects with the aim of helping to determine the pathogenicity of VUS in ABCD1. Fibroblasts from 36 male patients with confirmed ALD, 26 healthy control subjects and 17 individuals without a fa

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Stradomska, T. J., J. Kubalska, R. Janas, and A. Tylki-Szymańska. "Reproductive function in men affected by X-linked adrenoleukodystrophy/adrenomyeloneuropathy." European Journal of Endocrinology 166, no. 2 (2012): 291–94. http://dx.doi.org/10.1530/eje-11-0490.

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BackgroundX-linked adrenoleukodystrophy (X-ALD) is the most frequent, severely neurodegenerative, clinically heterogeneous peroxisomal disorder, the signs of which are a consequence of myelin, adrenal cortex, and testes impairment.ObjectiveWe studied testosterone, LH, and FSH levels in X-ALD/adrenomyeloneuropathy (AMN) patients. We evaluate the ability to procreate of these patients by analysis of pedigree and family screening by detection of very long-chain fatty acid (VLCFA) levels.Subject and methodsSeventeen patients with X-ALD/AMN (16 with AMN and one asymptomatic) aged 24–48 (mean±s.d.,

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Street, J. M., H. Singh та A. Poulos. "Metabolism of saturated and polyunsaturated very-long-chain fatty acids in fibroblasts from patients with defects in peroxisomal β-oxidation". Biochemical Journal 269, № 3 (1990): 671–77. http://dx.doi.org/10.1042/bj2690671.

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The metabolism of [1-14C]lignoceric acid (C24:0) and [1-14C]tetracosatetraenoic acid (C24:4, n-6) was studied in normal skin fibroblast cultures and in cultures from patients with defects in peroxisomal β-oxidation (but normal peroxisomal numbers). Cells from X-linked adrenoleukodystrophy (ALD) patients with a presumed defect in a peroxisomal acyl-CoA synthetase, specific for fatty acids of carbon chain lengths greater than 22 (very-long-chain fatty acids; VLCFA), showed a relatively normal production of radiolabelled CO2 and water-soluble metabolites from [1-14C]C24:0. However, the products o

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Villoria-González, Andrea, Bettina Zierfuss, Patricia Parzer та ін. "Efficacy of HDAC Inhibitors in Driving Peroxisomal β-Oxidation and Immune Responses in Human Macrophages: Implications for Neuroinflammatory Disorders". Biomolecules 13, № 12 (2023): 1696. http://dx.doi.org/10.3390/biom13121696.

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Elevated levels of saturated very long-chain fatty acids (VLCFAs) in cell membranes and secreted lipoparticles have been associated with neurotoxicity and, therefore, require tight regulation. Excessive VLCFAs are imported into peroxisomes for degradation by β-oxidation. Impaired VLCFA catabolism due to primary or secondary peroxisomal alterations is featured in neurodegenerative and neuroinflammatory disorders such as X-linked adrenoleukodystrophy and multiple sclerosis (MS). Here, we identified that healthy human macrophages upregulate the peroxisomal genes involved in β-oxidation during mye

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Hama, Kotaro, Yuko Fujiwara, Shigeo Takashima, et al. "Hexacosenoyl-CoA is the most abundant very long-chain acyl-CoA in ATP binding cassette transporter D1-deficient cells." Journal of Lipid Research 61, no. 4 (2020): 523–36. http://dx.doi.org/10.1194/jlr.p119000325.

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X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder caused by deleterious mutations in the ABCD1 gene. The ABCD1 protein transports very long-chain FAs (VLCFAs) from the cytosol into the peroxisome where the VLCFAs are degraded through β-oxidation. ABCD1 dysfunction leads to VLCFA accumulation in individuals with X-ALD. FAs are activated by esterification to CoA before metabolic utilization. However, the intracellular pools and metabolic profiles of individual acyl-CoA esters have not been fully analyzed. In this study, we profiled the acyl-CoA species in fibroblasts from X-ALD pati

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Vanderlinde, Elizabeth M., Artur Muszyński, Joe J. Harrison, et al. "Rhizobium leguminosarum biovar viciae 3841, deficient in 27-hydroxyoctacosanoate-modified lipopolysaccharide, is impaired in desiccation tolerance, biofilm formation and motility." Microbiology 155, no. 9 (2009): 3055–69. http://dx.doi.org/10.1099/mic.0.025031-0.

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The lipopolysaccharide (LPS) of the Gram-negative legume symbiont Rhizobium leguminosarum biovar viciae 3841 contains several unique modifications, including the addition of a 27-hydroxyoctacosanoic acid (27OHC28 : 0), also termed the very long chain fatty acid (VLCFA), attached at the 2′ position of lipid A. A transposon mutant that lacks expression of two putative 3-oxo-acyl [acyl-carrier protein] synthase II genes, fabF1 and fabF2, from the VLCFA biosynthetic cluster, was isolated and characterized. MS indicated that the lipid A of the mutant lacked the VLCFA modification, and sodium deoxyc

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Takahashi, Hideomi, Aiko Ohki, Mitsuru Kanzaki, et al. "Very-Long-Chain Fatty Acid Biosynthesis is Inhibited by Cafenstrole, N,N-Diethyl-3-mesitylsulfonyl-1H-1,2,4-triazole-1-carboxamide and Its Analogs." Zeitschrift für Naturforschung C 56, no. 9-10 (2001): 781–86. http://dx.doi.org/10.1515/znc-2001-9-1016.

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AbstractThe rice herbicide cafenstrole and its analogs inhibited the incorporation of [1-14C]-oleate and [2-14C]-malonate into very-long-chain fatty acids (VLCFAs), using Scenedesmus cells and leek microsomes from Allium porrum. Although the precise mode of interaction of cafenstrole at the molecular level is not completely clarified by the present study, it is concluded that cafenstrole acts as a specific inhibitor of the microsomal elongase enzyme involved in the biosynthesis of fatty acids with alkyl chains longer than C18. For a strong VLCFA biosynthesis inhibition an -SO2- linkage of the

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Hall, N. A., G. W. Lynes, and N. M. Hjelm. "Ratios for very-long-chain fatty acids in plasma of subjects with peroxisomal disorders, as determined by HPLC and validated by gas chromatography-mass spectrometry." Clinical Chemistry 34, no. 6 (1988): 1041–45. http://dx.doi.org/10.1093/clinchem/34.6.1041.

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Abstract We describe an HPLC method for measurement of ratios of concentrations of very-long-chain fatty acids (VLCFA) in plasma. The method, which involves ultraviolet detection of p-bromophenacyl derivatives of fatty acids, is validated by comparison with a gas chromatographic-mass spectrometric (GC-MS) method. The correlation between the ratios of 24-carbon fatty acids to 22-carbon fatty acids (C24/C22) estimated by the two methods was close (r = 0.976) as was the correlation for the C26/C22 ratios (r = 0.947). Increased VLCFA ratios could be demonstrated by either technique in patients wit

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Buda, Agnieszka, Sonja Forss-Petter, Rong Hua, et al. "ABCD1 Transporter Deficiency Results in Altered Cholesterol Homeostasis." Biomolecules 13, no. 9 (2023): 1333. http://dx.doi.org/10.3390/biom13091333.

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X-linked adrenoleukodystrophy (X-ALD), the most common peroxisomal disorder, is caused by mutations in the peroxisomal transporter ABCD1, resulting in the accumulation of very long-chain fatty acids (VLCFA). Strongly affected cell types, such as oligodendrocytes, adrenocortical cells and macrophages, exhibit high cholesterol turnover. Here, we investigated how ABCD1 deficiency affects cholesterol metabolism in human X-ALD patient-derived fibroblasts and CNS tissues of Abcd1-deficient mice. Lipidome analyses revealed increased levels of cholesterol esters (CE), containing both saturated VLCFA a

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Spreghini, Maria Rita, Nicoletta Gianni, Tommaso Todisco, Cristiano Rizzo, Marco Cappa, and Melania Manco. "Nutritional Counseling and Mediterranean Diet in Adrenoleukodystrophy: A Real-Life Experience." Nutrients 16, no. 19 (2024): 3341. http://dx.doi.org/10.3390/nu16193341.

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Background/Objectives: Adrenoleukodystrophy (X-ALD) is a metabolic disorder caused by dysfunctional peroxisomal beta-oxidation of very-long-chain fatty acids (VLCFAs). A VLCFA-restricted Mediterranean diet has been proposed for patients and carriers to reduce daily VLCFA intake. Methods: We retrospectively evaluated plasma VLCFAs in a cohort of 36 patients and 20 carriers at baseline and after 1 year of restricted diet. Results: At T1, compliant adult patients had significantly lower C26:0 levels [1.7 (1.2) vs. 2.5 µmol/L (1.7), p < 0.05], C26:0/C22:0 ratio [0.04 (0.02) vs. 0.06 (0.03), p &

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Stradomska, T. J., and A. Tylki-Szymańska. "Decreasing serum VLCFA levels in ageing X-ALD female carriers." Journal of Inherited Metabolic Disease 24, no. 8 (2001): 851–57. http://dx.doi.org/10.1023/a:1013992224811.

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Soltani, Nader, Lynette R. Brown, and Peter H. Sikkema. "Weed Control in Corn and Soybean with Group 15 (VLCFA Inhibitor) Herbicides Applied Preemergence." International Journal of Agronomy 2019 (April 7, 2019): 1–7. http://dx.doi.org/10.1155/2019/8159671.

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Limited information exists on the efficacy of pethoxamid for annual grass and broadleaf control in corn and soybean in Ontario. A total of 10 field experiments (5 with corn and 5 with soybean) were conducted during 2015 to 2017 in Ontario, Canada, to compare the weed control efficacy of dimethenamid-P at 544 g·ai·ha−1, pethoxamid at 840 g·ai·ha−1, pyroxasulfone at 100 g·ai·ha−1, and S-metolachlor at 1050 g·ai·ha−1 applied preemergence (PRE). Reduced weed interference with pyroxasulfone and dimethenamid-P resulted in corn yield that was similar to the weed-free control; however, weed interferen

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Robinson, B. S., D. W. Johnson, and A. Poulos. "Metabolism of hexacosatetraenoic acid (C26:4,n-6) in immature rat brain." Biochemical Journal 267, no. 2 (1990): 561–64. http://dx.doi.org/10.1042/bj2670561.

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Rat brain was recently found to contain polyenoic very-long-chain fatty acids (VLCFA) belonging to the n-3 and n-6 series with four, five and six double bonds and even-carbon chain lengths from 24 to 38 [Robinson, Johnson & Poulos (1990) Biochem. J. 265, 763-767]. In the present paper, the metabolism in vivo of hexacosatetraenoic acid (C26:4,n-6) was studied in neonatal rat brain. Rats were injected intracerebrally with [1-14C]C26:4,n-6 and the labelled metabolites were examined after 4 h. Radioactivity was detected mainly in non-esterified fatty acids, with smaller amounts in other neutra

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Dohr, Katrin A., Silvija Tokic, Magdalena Gastager-Ehgartner, et al. "Two Single Nucleotide Deletions in the ABCD1 Gene Causing Distinct Phenotypes of X-Linked Adrenoleukodystrophy." International Journal of Molecular Sciences 24, no. 6 (2023): 5957. http://dx.doi.org/10.3390/ijms24065957.

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X-linked adrenoleukodystrophy (X-ALD) is a rare inborn error of the peroxisomal metabolism caused by pathologic variants in the ATP-binding cassette transporter type D, member 1 (ABCD1) gene located on the X-chromosome. ABCD1 protein, also known as adrenoleukodystrophy protein, is responsible for transport of the very long chain fatty acids (VLCFA) from cytoplasm into the peroxisomes. Therefore, altered function or lack of the ABCD1 protein leads to accumulation of VLCFA in various tissues and blood plasma leading to either rapidly progressive leukodystrophy (cerebral ALD), progressive adrenom

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Bertucci, Matthew B., Michael Fogleman, and Jason K. Norsworthy. "Efficacy of fall-applied residual herbicides on weedy rice control in rice (Oryza sativa L.)." Weed Technology 33, no. 03 (2019): 441–47. http://dx.doi.org/10.1017/wet.2019.24.

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AbstractField experiments were initiated near Colt, AR, in the fall of 2016 and continued through the summer of 2018 to evaluate rice tolerance and weedy (or red) rice control after fall-applied very-long-chain fatty acid (VLCFA)-inhibiting herbicides. A split-plot design was used for the experiment, with the whole-plot factor being winter condition (flooded or non-flooded) and the split-plot factors being herbicide and rate. Herbicide treatments included acetochlor, dimethenamid-P, pethoxamid, pyroxasulfone, andS-metolachlor applied at 1,050, 525, 420, 205, and 1,070 g ai ha−1and at 2,100, 1,

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Stradomska, Tresa J. "Choroby peroksysomalne." Postępy Biochemii 64, no. 4 (2018): 359–67. http://dx.doi.org/10.18388/pb.2018_150.

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Peroksysomy to wielofunkcyjne organelle, które spełniają kluczową rolę w licznych procesach biochemicznych dostosowując się dynamicznie do aktualnych wymogów fizjologicznych komórki. Zaburzenie struktury peroksysomów na skutek mutacji i dysfunkcji genów PEX lub innych genów kodujących białka biogenezy czy pojedyncze peroksysomalne białka funkcyjne stanowi podłoże patogenetyczne chorób peroksysomalnych. Proces β-oksydacji bardzo długołańcuchowych kwasów tłuszczowych (VLCFA) jest unikalnym szlakiem metabolicznym zlokalizowanym wyłącznie w peroksysomie. To warunkuje, że VLCFA jest głównym biomark

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Kohlwein, Sepp D., Sandra Eder, Chan-Seok Oh, et al. "Tsc13p Is Required for Fatty Acid Elongation and Localizes to a Novel Structure at the Nuclear-Vacuolar Interface inSaccharomyces cerevisiae." Molecular and Cellular Biology 21, no. 1 (2001): 109–25. http://dx.doi.org/10.1128/mcb.21.1.109-125.2001.

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ABSTRACT The TSC13/YDL015c gene was identified in a screen for suppressors of the calcium sensitivity of csg2Δ mutants that are defective in sphingolipid synthesis. The fatty acid moiety of sphingolipids in Saccharomyces cerevisiae is a very long chain fatty acid (VLCFA) that is synthesized by a microsomal enzyme system that lengthens the palmitate produced by cytosolic fatty acid synthase by two carbon units in each cycle of elongation. TheTSC13 gene encodes a protein required for elongation, possibly the enoyl reductase that catalyzes the last step in each cycle of elongation. The tsc13 muta

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Erdbrügger, Pia, and Florian Fröhlich. "The role of very long chain fatty acids in yeast physiology and human diseases." Biological Chemistry 402, no. 1 (2020): 25–38. http://dx.doi.org/10.1515/hsz-2020-0234.

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AbstractFatty acids (FAs) are a highly diverse class of molecules that can have variable chain length, number of double bonds and hydroxylation sites. FAs with 22 or more carbon atoms are described as very long chain fatty acids (VLCFAs). VLCFAs are synthesized in the endoplasmic reticulum (ER) through a four-step elongation cycle by membrane embedded enzymes. VLCFAs are precursors for the synthesis of sphingolipids (SLs) and glycerophospholipids. Besides their role as lipid constituents, VLCFAs are also found as precursors of lipid mediators. Mis-regulation of VLCFA metabolism can result in a

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Ni, Fei, Mao Yang, Jun Chen, et al. "BnUC1 Is a Key Regulator of Epidermal Wax Biosynthesis and Lipid Transport in Brassica napus." International Journal of Molecular Sciences 25, no. 17 (2024): 9533. http://dx.doi.org/10.3390/ijms25179533.

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The bHLH (basic helix–loop–helix) transcription factor AtCFLAP2 regulates epidermal wax accumulation, but the underlying molecular mechanism remains unknown. We obtained BnUC1mut (BnaA05g18250D homologous to AtCFLAP2) from a Brassica napus mutant with up-curling leaves (Bnuc1) and epidermal wax deficiency via map-based cloning. BnUC1mut contains a point mutation (N200S) in the conserved dimerization domain. Overexpressing BnUC1mut in ZS11 (Zhongshuang11) significantly decreased the leaf epidermal wax content, resulting in up-curled and glossy leaves. In contrast, knocking out BnUC1mut in ZS11-

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Lipiński, Patryk, Piotr Stawiński, Małgorzata Rydzanicz, et al. "Mild Zellweger syndrome due to functionally confirmed novel PEX1 variants." Journal of Applied Genetics 61, no. 1 (2019): 87–91. http://dx.doi.org/10.1007/s13353-019-00523-w.

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Abstract Zellweger spectrum disorders (ZSD) constitute a group of rare autosomal recessive disorders characterized by a defect in peroxisome biogenesis due to mutations in one of 13 PEX genes. The broad clinical heterogeneity especially in late-onset presenting patients and a mild phenotype complicates and delays the diagnostic process. Here, we report a case of mild ZSD, due to novel PEX1 variants. The patient presented with an early hearing loss, bilateral cataracts, and leukodystrophy on magnetic resonance (MR) images. Normal results of serum very-long-chain fatty acids (VLCFA) and phytanic

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Vargas, Carmen R., Alethéa G. Barschak, Daniella M. Coelho, et al. "Clinical and biochemical findings in 7 patients with X-linked adrenoleukodystrophy treated with Lorenzo's Oil." Genetics and Molecular Biology 23, no. 4 (2000): 697–701. http://dx.doi.org/10.1590/s1415-47572000000400001.

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X-Linked adrenoleukodystrophy (X-ALD) is a hereditary disorder of the peroxisomal metabolism biochemically characterized by the accumulation of very long chain fatty acids (VLCFA) in tissues and biological fluids. The major accumulated acids are hexacosanoic acid (C26:0) and tetracosanoic acid (C24:0). The disorder is characterized clinically by central and peripheral demyelination and adrenal insufficiency closely related to the accumulation of fatty acids. The incidence of X-ALD is estimated to be 1:25,000 males. At least six phenotypes can be distinguished. The most common phenotypes are ch

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Hay, Marshall M., Jeffrey J. Albers, J. Anita Dille, and Dallas E. Peterson. "Control of Atrazine-Resistant Palmer Amaranth (Amaranthus palmeri) in Double-Crop Grain Sorghum." Weed Technology 33, no. 1 (2019): 115–22. http://dx.doi.org/10.1017/wet.2018.102.

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AbstractDouble-crop grain sorghum after winter wheat harvest is a common cropping system in the southern plains region. Palmer amaranth is a troublesome weed in double-crop grain sorghum in Kansas. Populations resistant to various herbicides (e.g., atrazine, glyphosate, metsulfuron, pyrasulfotole) have made Palmer amaranth management even more difficult for producers. To evaluate control of atrazine-resistant and atrazine-susceptible Palmer amaranth in double-crop grain sorghum, we assessed 14 herbicide programs, of which 8 were PRE only and 6 were PRE followed by (fb) POST applications. Visib

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Gagianone, R. B., and M. G. L. Ribeiro. "USING SCIENTIFIC PAPERS TO STIMULATE THE STUDY OF BIOCHEMISTRY AND THE UNDERSTANDING OF SCIENTIFIC KNOWLEDGE CONSTRUCTION: THE RESEARCH ON ADRENOLEUKODYSTROPHY." Revista de Ensino de Bioquímica 13 (August 24, 2015): 6. http://dx.doi.org/10.16923/reb.v13i2.574.

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Introduction The X-linked adrenoleukodystrophy (X-ALD) is characterized by mutations in very long chain fatty acids (VLCFA) peroxisome transporter, leading to VLCFA accumulation in myelin sheath. In the 70’s and 80’s it was hypothesized that X-ALD is caused by enzymatic deficits in FA-coenzyme A connection, VLCFA degradation or FA elongation. The latter enabled Lorenzo’s oil (LO) treatment, which became famous by the homonym movie. The apparent initial therapy effectiveness lead to LO administration in many patients, although with biochemical knowledge progress its relevance has been questione

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Strom, Seth A., Lisa C. Gonzini, Charlie Mitsdarfer, Adam S. Davis, Dean E. Riechers, and Aaron G. Hager. "Characterization of multiple herbicide–resistant waterhemp (Amaranthus tuberculatus) populations from Illinois to VLCFA-inhibiting herbicides." Weed Science 67, no. 4 (2019): 369–79. http://dx.doi.org/10.1017/wsc.2019.13.

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AbstractField experiments were conducted in 2016 and 2017 in Champaign County, IL, to study a waterhemp [Amaranthus tuberculatus (Moq.) J. D. Sauer] population (CHR) resistant to 2,4-D and 4-hydroxyphenylpyruvate dioxygenase (HPPD)-, photosystem II–, acetolactate synthase (ALS)-, and protoporphyrinogen oxidase–inhibiting herbicides. Two field experiments were designed to investigate the efficacy of very-long-chain fatty-acid (VLCFA)-inhibiting herbicides, including a comparison of active ingredients at labeled use rates and a rate titration experiment. Amaranthus tuberculatus density and contr

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Zheng, Huan, Yueting Liang, Ben Hong, et al. "Genome-Scale Analysis of the Grapevine KCS Genes Reveals Its Potential Role in Male Sterility." International Journal of Molecular Sciences 24, no. 7 (2023): 6510. http://dx.doi.org/10.3390/ijms24076510.

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Very long-chain fatty acid (VLCFA) synthesis in plants, is primarily rate-limited by the enzyme 3-ketoacyl CoA synthase (KCS), which also controls the rate and carbon chain length of VLCFA synthesis. Disruption of VLCFA during pollen development, may affect the pollen wall formation and ultimately lead to male sterility. Our study identified 24 grapevine KCS (VvKCS) genes and provided new names based on their relative chromosome distribution. Based on sequence alignment and phylogenetic investigation, these genes were grouped into seven subgroups, members of the same subgroup having similar mo

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Liu, Jinxin, Xin Wang, Di Huang, Yuna Qi, Lei Xu, and Yankun Shao. "A novel ABCD1 gene mutation causes adrenomyeloneuropathy presenting with spastic paraplegia: A case report." Medicine 103, no. 16 (2024): e37874. http://dx.doi.org/10.1097/md.0000000000037874.

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Rationale: X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene leading to very long chain fatty acid (VLCFA) accumulation. The disease demonstrates a spectrum of phenotypes including adrenomyeloneuropathy (AMN). We aimed to identify the genetic basis of disease in a patient presenting with AMN features in order to confirm the diagnosis, expand genetic knowledge of ABCD1 mutations, and elucidate potential genotype-phenotype associations to inform management. Patient concerns: A 29-year-old male presented with a 4-year history of progressive spastic paraplegia, weaknes

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Jawasreh, Khaleel, Dana Khrais, Muhammad datt, et al. "Fatty acids profile in Awassi sheep milk affected by some genes’ single and combined effects." Journal of Advanced Veterinary and Animal Research 12, no. 1 (2025): 238. https://doi.org/10.5455/javar.2025.l891.

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Objective: This study intended to figure out the effects of fixed effects and genes such as beta-lactoglobulin (β-LG), prolactin (PRL), annexin A9 (ANXA9), and acetyl-CoA acyltransferase 2 (ACAA2) on the structure of fatty milk acids in Awassi ewes, as well as any potential genotype-genotype interactions. Materials and Methods: Fatty acid (FA) profile and other milk components were examined from 116 Awassi ewes in total. Polymerase chain reaction (PCR) was used to extract and genotype their DNA, and either sequencing or restriction fragment length polymorphism (RFLP) analysis came next. Result

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Streck, Sibylle, K. Winnefeld, Iris Maurer, and H. P. Volz. "Die quantitative Bestimmung ausgewählter langkettiger Fettsäuren und sehr langkettiger Fettsäuren (VLCFA) im Serum. The Quantitative Determination of Selected Long Chain and Very Long Chain Fatty Acids (VLCFA) in Serum." LaboratoriumsMedizin 24, no. 8 (2000): 373–76. http://dx.doi.org/10.1515/labm.2000.24.8.373.

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Shon, Jinyoung, Yerim Han, and Yoon Jung Park. "Effects of Dietary Fat to Carbohydrate Ratio on Obesity Risk Depending on Genotypes of Circadian Genes." Nutrients 14, no. 3 (2022): 478. http://dx.doi.org/10.3390/nu14030478.

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Although the impacts of macronutrients and the circadian clock on obesity have been reported, the interactions between macronutrient distribution and circadian genes are unclear. The aim of this study was to explore macronutrient intake patterns in the Korean population and associations between the patterns and circadian gene variants and obesity. After applying the criteria, 5343 subjects (51.6% male, mean age 49.4 ± 7.3 years) from the Korean Genome and Epidemiology Study data and nine variants in seven circadian genes were analyzed. We defined macronutrient intake patterns by tertiles of th

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Marlow, Victoria L., Andreas F. Haag, Hajime Kobayashi, et al. "Essential Role for the BacA Protein in the Uptake of a Truncated Eukaryotic Peptide in Sinorhizobium meliloti." Journal of Bacteriology 191, no. 5 (2008): 1519–27. http://dx.doi.org/10.1128/jb.01661-08.

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ABSTRACT The inner membrane BacA protein is essential for the establishment of chronic intracellular infections by Sinorhizobium meliloti and Brucella abortus within plant and mammalian hosts, respectively. In their free-living state, S. meliloti and B. abortus mutants lacking BacA have reductions in their outer membrane lipid A very-long-chain fatty acid (VLCFA) contents and exhibit low-level resistance to the glycopeptide bleomycin in comparison to their respective parent strains. In this paper we investigate the hypothesis that BacA is involved in peptide uptake in S. meliloti. We determine

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Song, Jinsoo, Yeon-Ho Kang, Sik Yoon, Churl-Hong Chun та Eun-Jung Jin. "HIF-1α:CRAT:miR-144-3p axis dysregulation promotes osteoarthritis chondrocyte apoptosis and VLCFA accumulation". Oncotarget 8, № 41 (2017): 69351–61. http://dx.doi.org/10.18632/oncotarget.20615.

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Doria, Margaux, Thomas Nury, Dominique Delmas, Thibault Moreau, Gérard Lizard, and Anne Vejux. "Protective function of autophagy during VLCFA-induced cytotoxicity in a neurodegenerative cell model." Free Radical Biology and Medicine 137 (June 2019): 46–58. http://dx.doi.org/10.1016/j.freeradbiomed.2019.04.016.

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Gong, Yi, Fiza Laheji, Anna Berenson, et al. "Peroxisome Metabolism Contributes to PIEZO2-Mediated Mechanical Allodynia." Cells 11, no. 11 (2022): 1842. http://dx.doi.org/10.3390/cells11111842.

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Mutations in the peroxisomal half-transporter ABCD1 cause X-linked adrenoleukodystrophy, resulting in elevated very long-chain fatty acids (VLCFA), progressive neurodegeneration and an associated pain syndrome that is poorly understood. In the nervous system of mice, we found ABCD1 expression to be highest in dorsal root ganglia (DRG), with satellite glial cells (SGCs) displaying higher expression than neurons. We subsequently examined sensory behavior and DRG pathophysiology in mice deficient in ABCD1 compared to wild-type mice. Beginning at 8 months of age, Abcd1−/y mice developed persistent

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Binczek, Erika, Britta Jenke, Barbara Holz, Robert Heinz Günter, Mario Thevis, and Wilhelm Stoffel. "Obesity resistance of the stearoyl-CoA desaturase-deficient (scd1 -/-) mouse results from disruption of the epidermal lipid barrier and adaptive thermoregulation." Biological Chemistry 388, no. 4 (2007): 405–18. http://dx.doi.org/10.1515/bc.2007.046.

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Abstract Targeted deletion of the stearoyl-CoA desaturase 1 gene (scd1) in mouse causes obesity resistance and a severe skin phenotype. Here, we demonstrate that SCD1 deficiency disrupts the epidermal lipid barrier and leads to uncontrolled transepidermal water loss, breakdown of adaptive thermoregulation and cold resistance, as well as a metabolic wasting syndrome. The loss of ω-hydroxylated very long-chain fatty acids (VLCFA) and ceramides substituted with ω-hydroxylated VLCFA covalently linked to corneocyte surface proteins leads to the disruption of the epidermal lipid barrier in scd1 -/-

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Song, Jinsoo, Yeon-Ho Kang, Sik Yoon, Churl-Hong Chun та Eun-Jung Jin. "Correction: HIF-1α:CRAT:miR-144-3p axis dysregulation promotes osteoarthritis chondrocyte apoptosis and VLCFA accumulation". Oncotarget 10, № 44 (2019): 4609–10. http://dx.doi.org/10.18632/oncotarget.27091.

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Clayton, P. T. "Clinical consequences of defects in peroxisomal β-oxidation". Biochemical Society Transactions 29, № 2 (2001): 298–305. http://dx.doi.org/10.1042/bst0290298.

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The disorders of peroxisomal β-oxidation, which have been well characterised at the molecular level, include defects of acyl-CoA oxidase, defects of the D-bifunctional protein (D-BP) (including specific defects of its enoyl-CoA hydratase and D-3-hydroxyacyl-CoA dehydrogenase components), defects of the very-long-chain fatty acid (VLCFA)-CoA importer [X-linked adrenoleukodystrophy (ALD)] and α-methylacyl-CoA racemase deficiency. A survey of the clinical consequences of these defects indicates that defects in the acyl-CoA oxidase and D-BP can produce neonatal hypotonia, seizures in early infancy

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