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Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD-/-) mouse to compensate a defective fatty acid β-oxidationThe pathobiology of splicingCombined defects in oxidative phosphorylation and fatty acid β-oxidation in mitochondrial diseaseA Mechanistic Review of Mitophagy and Its Role in Protection against Alcoholic Liver DiseaseMitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studiesPantothenate kinase 1 is required to support the metabolic transition from the fed to the fasted stateFatty acids-stress attenuates gluconeogenesis induction and glucose production in primary hepatocytesGbb/BMP signaling is required to maintain energy homeostasis in Drosophila.Mitochondrial trifunctional protein deficiency due to HADHB gene mutation in a Chinese family.A novel mutation of the ACADM gene (c.145C>G) associated with the common c.985A>G mutation on the other ACADM allele causes mild MCAD deficiency: a case reportBirth Prevalence of Fatty Acid β-Oxidation Disorders in Iberia.Infant with cardiomyopathy: When to suspect inborn errors of metabolism?Carnitine Deficiency and Pregnancy.Rhabdomyolysis in a patient treated with linezolid for extensively drug-resistant tuberculosis.Metabolic Encephalopathy and Lipid Storage Myopathy Associated with a Presumptive Mitochondrial Fatty Acid Oxidation Defect in a Dog.Unanswered questions in Friedreich ataxia.Fatty acid degradation plays an essential role in proliferation of mouse female primordial germ cells via the p53-dependent cell cycle regulation.Peroxisome-mitochondria interplay and disease.Current Applications of Chromatographic Methods in the Study of Human Body Fluids for Diagnosing Disorders.PAQR-2 may be a regulator of membrane fluidity during cold adaptation.Metabolic adaptations of Leishmania donovani in relation to differentiation, drug resistance, and drug pressure.ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome.Identification of human plasma metabolites exhibiting time-of-day variation using an untargeted liquid chromatography-mass spectrometry metabolomic approach.Renal response to short- and long-term exercise in very-long-chain acyl-CoA dehydrogenase-deficient (VLCAD(-/-)) mice.De novo fatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids.Caspase-1 cleaves PPARγ for potentiating the pro-tumor action of TAMs.Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient mice.Clinical and biological features at diagnosis in mitochondrial fatty acid beta-oxidation defects: a French pediatric study of 187 patients.Polycyclic aromatic hydrocarbons (PAHs) reduce hepatic β-oxidation of fatty acids in chick embryos.Mitochondrial fatty acid biosynthesis and muscle fibre plasticity in very long-chain acyl-CoA dehydrogenase-deficient mice.Infant with hepatomegaly and hypoglycemia: A setting for fatty acid oxidation defects.Serum vitamin C levels modulate the lifespan and endoplasmic reticulum stress response pathways in mice synthesizing a nonfunctional mutant WRN protein.Open-label clinical trial of bezafibrate treatment in patients with fatty acid oxidation disorders in Japan.Mitochondrial Fatty Acid Oxidation Disorders Associated with Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency.Cryo-EM structure of human mitochondrial trifunctional protein
P2860
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P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on September 2008
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Mitochondrial fatty-acid oxidation disorders.
@en
Mitochondrial fatty-acid oxidation disorders.
@nl
type
label
Mitochondrial fatty-acid oxidation disorders.
@en
Mitochondrial fatty-acid oxidation disorders.
@nl
prefLabel
Mitochondrial fatty-acid oxidation disorders.
@en
Mitochondrial fatty-acid oxidation disorders.
@nl
P1476
Mitochondrial fatty-acid oxidation disorders.
@en
P2093
Michelle Kompare
William B Rizzo
P304
P356
10.1016/J.SPEN.2008.05.008
P577
2008-09-01T00:00:00Z