Bibliografías temáticas / VLFCA

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Literatura académica sobre el tema "VLFCA"

Autor: Grafiati
Publicado: 12 de octubre de 2024
Última modificación: 31 de julio de 2025

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Artículos de revistas sobre el tema "VLFCA"

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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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Tesis sobre el tema "VLFCA"

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Le, ruyet Claire. "Investigating herbicide targets in the very long-chain fatty acid biosynthesis pathway." Electronic Thesis or Diss., Bordeaux, 2024. http://www.theses.fr/2024BORD0125.

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Les acides gras à très longue chaîne (AGTLC) sont des molécules essentielles avec des fonctions physiologiques et structurales importantes chez les plantes. Ces acides gras, qui ont des chaînes acyles de 20 carbones ou plus, peuvent être modifiés en composants de lipides clés tels que les phospholipides et les sphingolipides des membranes, les triacylglycérols (TAG) dans les graines, les cires cuticulaires dans l'épiderme et la subérine dans les racines. L'élongation des AGTLC est réalisée par le complexe d'élongation des acides gras (FAE complex). Ce complexe comprend quatre enzymes distincte
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Hay, Marshall Mark. "Control of Palmer amaranth (Amaranthus palmeri) and common waterhemp (Amaranthus rudis) in double crop soybean and with very long chain fatty acid inhibitor herbicides." Thesis, Kansas State University, 2017. http://hdl.handle.net/2097/35489.

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Master of Science<br>Department of Agronomy<br>Dallas E. Peterson<br>During 2015 and 2016, five site years of research were implemented in double crop soybean after winter wheat at experiment fields in Kansas near Manhattan, Hutchinson, and Ottawa to assess various non-glyphosate herbicide treatments at three different application timings for control of Palmer amaranth (Amaranthus palmeri S. Wats.) and common waterhemp (Amaranthus rudis Sauer). Spring-post (SP) treatments with residual control of Palmer amaranth and waterhemp were applied in the winter wheat at Feekes 4 and resulted in less t
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Morineau, Céline. "Caractérisation d'un nouveau membre du complexe d'élongation des acides gras chez Arabidopsis thaliana : intéractions métaboliques et régulation développementale." Thesis, Paris 11, 2014. http://www.theses.fr/2014PA112420.

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Les acides gras à très longues chaine (VLCFA) sont essentiels dans le développement, particulièrement dans les mécanismes de trafic vésiculaires, de différenciation et division cellulaire. Cependant, le rôle de ces VLCFA dans ces différents processus chez les plantes n’est pas encore bien compris. Afin d’identifier de nouveaux acteurs associés à la biosynthèse ou la fonction des VLCFA, un crible suppresseur multicopies a été réalisé dans un mutant d’élongation des VLCFA de levure. La perte de l’activité déshydratase PHS1 chez la levure et de PASTICCINO2 chez les plantes perturbe la croissance
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Marlow, Victoria L. "Investigating bacterial factors important for the sinorhizobium meliloti-legume symbiosis." Thesis, University of Edinburgh, 2009. http://hdl.handle.net/1842/4331.

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In both the legume symbiont Sinorhizobium meliloti and the mammalian pathogen Brucella abortus, the inner membrane BacA protein is essential for host persistence. In free-living S. meliloti and B. abortus loss of the BacA protein also results in an increased resistance to the glycopeptide bleomycin and a ~ 50% decrease in the lipopolysaccharide (LPS) very-long-chain-fatty-acid (VLCFA) content. Consequently, it was proposed that BacA may be involved in transport of peptides into the cell and/or that BacA may be involved in the VLCFA modification of the LPS. During this work it was determined th
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El, Hajj Hammam. "Altérations mitochondriales et processus inflammatoire dans la déficience en acyl- Coenzyme A oxydase 1 peroxysomale." Phd thesis, Université de Bourgogne, 2012. http://tel.archives-ouvertes.fr/tel-00995795.

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L'acyl-CoA oxydase 1 (ACOX1) est l'enzyme qui catalyse la première étape de la voie classique de la β-oxydation peroxysomale. Cette voie catabolise exclusivement les acides gras à très longue chaîne (AGTLC). Chez l'homme, la déficience en ACOX1 est à l'origine de la pseudo adrénoleucodystrophie néonatale (P-NALD), une maladie neurodégénérative rare caractérisée par une accumulation des AGTLC dans le plasma et les tissus, une hépatomégalie, un retard du développement moteur et une démyélinisation de la matière blanche cérébrale. Chez la souris, l'extinction du gène Acox1 provoque une accumulati
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Van, der Linde Wilhelmina Johanna. "The metabolic profile of phenylbutyric acid and its antioxidant capacity in vervet monkeys / Wilhelmina Johanna van der Linde." Thesis, North-West University, 2010. http://hdl.handle.net/10394/4916.

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X–linked adrenoleukodystrophy (X–ALD) is the most common peroxisomal enzyme deficiency disorder, characterized by inborn mutations in the ABCD1 gene, an ATP–binding cassette (ABC) half–transporter. The ABCD1 gene encodes the adrenoleukodystrophy protein (ALDP), the transporter for the very–long–chain fatty acids (VLCFA; C > 22:0) from the cytosol into the peroxisomes to enter the peroxisomal B–oxidation pathway. The diagnostic disease marker is the elevated levels of VLCFAs which accumulate in different tissues and body fluids, leading to inflammatory demyelination, neuro–deterioration and adr
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Rosa, Ricardo Manuel Balixa da. "Unravel the functions of Acbd5 through the generation and characterization of mouse mutants." Master's thesis, 2019. http://hdl.handle.net/1822/65492.

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Dissertação de mestardo em Applied Biochemistry Specialization (área de especialização em Biomedicine)<br>Lipids are the major class of macromolecules present in the brain, so mutations in proteins that are somehow related with lipid metabolism may cause several disorders in the nervous system. Peroxisome is one vital organelle in lipid metabolism, catalyzing anabolic and catabolic processes, such as fatty acid oxidation and plasmalogen biosynthesis, crucial for myelination. Acyl-CoA binding domain 5 (ACBD5) is a peroxisomal membranar protein implicated in the internalization of very long chai
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Libros sobre el tema "VLFCA"

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van Geel, Björn M., Marc Engelen, and Stephan Kemp. X-linked Adrenoleukodystrophy. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0061.

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X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disorder. Hallmarks are increased levels of plasma very long-chain fatty acids (VLCFA), mutations in the ABCD1 gene, impaired function of ALD-protein and, consequently, decreased import of VLCFA-CoA esters in peroxisomes and VLCFA beta-oxidation. Cerebral demyelination and axonal degeneration of the spinal cord are the main causes of neurological deficits. Endocrine dysfunction, particularly adrenocortical insufficiency, is very frequent. Based upon the age of onset of symptoms and the organs most severely affected, several
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Vaz, Frédéric M., and Ronald J. A. Wanders. Interpretation of Very-Long-Chain Fatty Acids Analysis Results. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0086.

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The analysis of very long-chain fatty acids (VLCFAs) is a crucial step in the diagnostic work-up of patients suspected to suffer from a peroxisomal disorder (PD) including X-linked adrenoleukodystrophy (X-ALD). Although different analytical procedures have been described for the analysis of VLCFAs, most laboratories use GC/MS-analysis as method of choice. As described below, VLCFA-analysis is a robust and reliable diagnostic method which is usually unequivocal except in rare cases.
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Capítulos de libros sobre el tema "VLFCA"

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Claude, Cassagne, Bessoule Jean-Jacques, Schneider Florence, et al. "Modulation of the Very-Long-Chain Fatty Acid (VLCFA) Formation in Leek." In Plant Lipid Metabolism. Springer Netherlands, 1995. http://dx.doi.org/10.1007/978-94-015-8394-7_32.

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"VLCFA." In Springer Reference Medizin. Springer Berlin Heidelberg, 2019. http://dx.doi.org/10.1007/978-3-662-48986-4_301707.

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Wierzbicki, Anthony S. "Disorders of peroxisomal metabolism in adults." In Oxford Textbook of Medicine. Oxford University Press, 2010. http://dx.doi.org/10.1093/med/9780199204854.003.1209_update_002.

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The peroxisome is a specialized organelle which employs molecular oxygen in the oxidation of complex organic molecules including lipids. Enzymatic pathways for the metabolism of fatty acids, including very long-chain fatty acids (VLCFA) enable this organelle to carry out β‎-oxidation in partnership with mitochondria. A peroxisomal pathway for isoprenoid lipids derived from chlorophyll, such as phytanic acid, utilizes ...
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Mendell, Jerry R. "Peroxisomal Defects: Refsum Disease and Adrenomyeloneuropathy." In Diagnosis and Management of Peripheral Nerve Disorders. Oxford University PressNew York, NY, 2001. http://dx.doi.org/10.1093/oso/9780195133011.003.0024.

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Abstract Peroxisomes are single-membrane-bound organelles, measuring about 0.2 to 0.5 µm in diameter. They lack DNA and have no defined internal cytoarchitecture. These organelles are found in all cells in the human body, especially the liver and kidney, but are absent from mature erythrocytes.1 Peroxisomes catalyze the 13 oxidation of fatty acids and related substrates. Those preferentially or exclusively oxidized in peroxisomes include very long chain fatty acids (VLCFA), polyunsaturated fatty acids, dicarboxylic fatty acids, prostaglandins, and the side chain of cholesterol. Peroxisomes als
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Moser, Hugo, and Nga Hong Brereton. "Adrenoleukodystrophy And Other Peroxisomal Disorders." In Pediatric Nutrition In Chronic Diseases And Developmental Disorders. Oxford University PressNew York, NY, 2005. http://dx.doi.org/10.1093/oso/9780195165647.003.0045.

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Abstract Genetically determined peroxisomal disorders are divided into two major categories: (1) disorders of peroxisome biogenesis, in which the organelle is not normally formed, and (2) disorders that involve a single peroxisomal enzyme. The biochemical and gene defects in each of these disorders have been defined. A list of the disorders with pertinent references follows: X-linked adrenoleukodystrophy (X-ALD) is the most common peroxisomal disorder. Its frequency is estimated to be 1:17,000 and approaches that of phenylketonuria. The gene defect involves ABCD1, a gene that has been mapped t
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Actas de conferencias sobre el tema "VLFCA"

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Van Duong, Binh, An Trong Nguyen, Chien Nhu Ha, Hong-Hanh Thi Duong, My-Linh Thi Tran, and Trong-Hop Do. "UIT-VLFC: Vietnamese Lipstick Feedbacks Corpus." In 2022 25th Conference of the Oriental COCOSDA International Committee for the Co-ordination and Standardisation of Speech Databases and Assessment Techniques (O-COCOSDA). IEEE, 2022. http://dx.doi.org/10.1109/o-cocosda202257103.2022.9997964.

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Vasconcelos, Matheus Felipe de Souza, Francisco Tomaz Meneses de Oliveira, Rafael Zini Moreira da Silva, and Alex Michel Daoud. "Neurological and adrenal insufficiency symptons in adult x-linked adrenoleukodystrophy: case report." In XIII Congresso Paulista de Neurologia. Zeppelini Editorial e Comunicação, 2021. http://dx.doi.org/10.5327/1516-3180.347.

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Context: X-linked adrenoleukodystrophy (X-ALD) is a rare genetic demyelinating disease caused by mutations in ABC1 gen associated with an impairment of beta- oxidation of very long chain fatty acids (VLCFA) in peroxisomes. It causes accumulation of VCLFA in tissues affecting majoritary the central nervous system, testicles and the adrenal córtex resulting in symptoms which provides restricted neurological prognosis and sequels. Methods: Specific data related of a clinical case through prontuary and complementary exams in a patient attended at Santa Casa de Misericórdia de São Paulo hospital. C
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