Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients
about
Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhoodIdentification and characterization of new long chain acyl-CoA dehydrogenasesStructural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenaseA new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiencyAbnormal fatty acid metabolism in childhood spinal muscular atrophyA novel approach to the characterization of substrate specificity in short/branched chain Acyl-CoA dehydrogenaseHADHB, HuR, and CP1 bind to the distal 3'-untranslated region of human renin mRNA and differentially modulate renin expressionPolyenephosphatidylcholine prevents alcoholic liver disease in PPARalpha-null mice through attenuation of increases in oxidative stressMitochondrial long chain fatty acid beta-oxidation in man and mouseStructural organization of the human short-chain acyl-CoA dehydrogenase geneClear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency.Interplay between oxidant species and energy metabolismCompared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.Dysregulation of very long chain acyl-CoA dehydrogenase coupled with lipid peroxidation.Myopathic form of very-long chain acyl-coa dehydrogenase deficiency: evidence for temperature-sensitive mild mutations in both mutant alleles in a Japanese girl.Nitric oxide regulates mitochondrial fatty acid metabolism through reversible protein S-nitrosylation.Nrf2 affects the efficiency of mitochondrial fatty acid oxidation.Diet-sensitive sources of reactive oxygen species in liver mitochondria: role of very long chain acyl-CoA dehydrogenases.Strategies for correcting very long chain acyl-CoA dehydrogenase deficiency.Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapyMolecular characterization of the mouse very-long-chain acyl-CoA dehydrogenase gene.Mitochondrial deficiency in Cockayne syndrome.Bezafibrate at clinically relevant doses decreases serum/liver triglycerides via down-regulation of sterol regulatory element-binding protein-1c in mice: a novel peroxisome proliferator-activated receptor alpha-independent mechanism.Fatty acid oxidation disorders as primary cause of sudden and unexpected death in infants and young children: an investigation performed on cultured fibroblasts from 79 children who died aged between 0-4 yearsMechanisms of disease: hypertrophic cardiomyopathy.Successful Treatment of Cardiomyopathy due to Very Long-Chain Acyl-CoA Dehydrogenase Deficiency: First Case Report from Oman with Literature Review.The emerging role of Nrf2 in mitochondrial functionBiochemical correction of very long-chain acyl-CoA dehydrogenase deficiency following adeno-associated virus gene therapy.Complementation analysis of carnitine palmitoyltransferase I and II defects.Mutation analysis of very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency: identification and characterization of mutant VLCAD cDNAs from four patients.Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunitsIdentification and characterization of an intracellular protein complex that binds fibroblast growth factor-2 in bovine brain.MCT oil-based diet reverses hypertrophic cardiomyopathy in a patient with very long chain acyl-coA dehydrogenase deficiency.Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency.Chronic ethanol consumption decreases serum sulfatide levels by suppressing hepatic cerebroside sulfotransferase expression in mice.Effect of bezafibrate on hepatic oxidative stress: comparison between conventional experimental doses and clinically-relevant doses in mice.Development of a new enzymatic diagnosis method for very-long-chain Acyl-CoA dehydrogenase deficiency by detecting 2-hexadecenoyl-CoA production and its application in tandem mass spectrometry-based selective screening and newborn screening in JapanPathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation.Adaptive remodeling of skeletal muscle energy metabolism in high-altitude hypoxia: Lessons from AltitudeOmics.Medium-Chain Acyl CoA Dehydrogenase: Evidence for Phosphorylation
P2860
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P2860
Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients
description
1995 nî lūn-bûn
@nan
1995 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
1995 թվականի հունիսին հրատարակված գիտական հոդված
@hy
1995年の論文
@ja
1995年論文
@yue
1995年論文
@zh-hant
1995年論文
@zh-hk
1995年論文
@zh-mo
1995年論文
@zh-tw
1995年论文
@wuu
name
Purification of human very-lon ...... s deficiency in seven patients
@ast
Purification of human very-lon ...... s deficiency in seven patients
@en
Purification of human very-lon ...... s deficiency in seven patients
@nl
type
label
Purification of human very-lon ...... s deficiency in seven patients
@ast
Purification of human very-lon ...... s deficiency in seven patients
@en
Purification of human very-lon ...... s deficiency in seven patients
@nl
prefLabel
Purification of human very-lon ...... s deficiency in seven patients
@ast
Purification of human very-lon ...... s deficiency in seven patients
@en
Purification of human very-lon ...... s deficiency in seven patients
@nl
P2093
P2860
P356
P1476
Purification of human very-lon ...... s deficiency in seven patients
@en
P2093
R I Kelley
S Ushikubo
S Yamaguchi
T Hashimoto
P2860
P304
P356
10.1172/JCI117947
P407
P577
1995-06-01T00:00:00Z