about
The minor C-allele of rs2014355 in ACADS is associated with reduced insulin release following an oral glucose loadMitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene.Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and managementMedium-chain acyl-CoA dehydrogenase deficiency in gene-targeted miceA general introduction to the biochemistry of mitochondrial fatty acid β-oxidationFatty Acid Beta-Oxidation Disorders: A Brief ReviewMitochondrial dysfunction in inflammatory bowel diseaseMitochondrial disease in childhood: nuclear encodedObservations regarding retinopathy in mitochondrial trifunctional protein deficienciesMitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studiesCarnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular reviewMitochondrial long chain fatty acid beta-oxidation in man and mouseCardiac hypertrophy in mice with long-chain acyl-CoA dehydrogenase or very long-chain acyl-CoA dehydrogenase deficiencyCarnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout micePantothenate kinase 1 is required to support the metabolic transition from the fed to the fasted stateCarnitine content and expression of mitochondrial beta-oxidation enzymes in placentas of wild-type (OCTN2(+/+)) and OCTN2 Null (OCTN2(-/-)) MiceMulti-organ abnormalities and mTORC1 activation in zebrafish model of multiple acyl-CoA dehydrogenase deficiencySpecificity in beta cell expression of L-3-hydroxyacyl-CoA dehydrogenase, short chain, and potential role in down-regulating insulin releaseMitochondrial Trifunctional Protein Defects: Clinical Implications and Therapeutic ApproachesInflammatory bowel diseaseAltered body composition and energy expenditure but normal glucose tolerance among humans with a long-chain fatty acid oxidation disorder.Kruppel-like factor 15 regulates skeletal muscle lipid flux and exercise adaptation.Biomarker discovery, disease classification, and similarity query processing on high-throughput MS/MS data of inborn errors of metabolism.Dynamic simulations on the mitochondrial fatty acid beta-oxidation network.Carnitine palmitoyltransferase 1B 531K allele carriers sustain a higher respiratory quotient after aerobic exercise, but β3-adrenoceptor 64R allele does not affect lipolysis: a human modelThioesterase superfamily member 2 (Them2) and phosphatidylcholine transfer protein (PC-TP) interact to promote fatty acid oxidation and control glucose utilization.Inborn errors of energy metabolism associated with myopathies.Describing hypoglycemia--definition or operational threshold?Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans.Mitochondrial topoisomerase I is critical for mitochondrial integrity and cellular energy metabolism.Carnitine deficiency in OCTN2-/- newborn mice leads to a severe gut and immune phenotype with widespread atrophy, apoptosis and a pro-inflammatory responseRole of carnitine in diseaseBiochemical characterization of L-carnitine dehydrogenases from Rhizobium sp. and Xanthomonas translucens.The functions of cardiolipin in cellular metabolism-potential modifiers of the Barth syndrome phenotypeDeletion of the mammalian INDY homolog mimics aspects of dietary restriction and protects against adiposity and insulin resistance in mice.Genetic defects in fatty acid beta-oxidation and acyl-CoA dehydrogenases. Molecular pathogenesis and genotype-phenotype relationships.Acyl-CoA Dehydrogenase Drives Heat Adaptation by Sequestering Fatty AcidsInduction of dsRNA-activated protein kinase links mitochondrial unfolded protein response to the pathogenesis of intestinal inflammation.Global Metabolomic Identification of Long-Term Dose-Dependent Urinary Biomarkers in Nonhuman Primates Exposed to Ionizing Radiation.
P2860
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P2860
description
2002 nî lūn-bûn
@nan
2002 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2002 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2002年の論文
@ja
2002年論文
@yue
2002年論文
@zh-hant
2002年論文
@zh-hk
2002年論文
@zh-mo
2002年論文
@zh-tw
2002年论文
@wuu
name
Fatty acid oxidation disorders.
@ast
Fatty acid oxidation disorders.
@en
Fatty acid oxidation disorders.
@nl
type
label
Fatty acid oxidation disorders.
@ast
Fatty acid oxidation disorders.
@en
Fatty acid oxidation disorders.
@nl
prefLabel
Fatty acid oxidation disorders.
@ast
Fatty acid oxidation disorders.
@en
Fatty acid oxidation disorders.
@nl
P2093
P1476
Fatty acid oxidation disorders.
@en
P2093
Dietrich Matern
Michael J Bennett
Piero Rinaldo
P304
P356
10.1146/ANNUREV.PHYSIOL.64.082201.154705
P577
2002-01-01T00:00:00Z