about
Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and managementA novel approach to the characterization of substrate specificity in short/branched chain Acyl-CoA dehydrogenaseLong-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: a molecular and biochemical reviewCarnitine content and expression of mitochondrial beta-oxidation enzymes in placentas of wild-type (OCTN2(+/+)) and OCTN2 Null (OCTN2(-/-)) MicePrenatal diagnosis of disorders of fatty acid transport and mitochondrial oxidation.Cardiac dysfunction and peri-weaning mortality in malonyl-coenzyme A decarboxylase (MCD) knockout mice as a consequence of restricting substrate plasticityEnergy metabolic reprogramming in the hypertrophied and early stage failing heart: a multisystems approach.Surprising? Perhaps not. Long-chain fatty acid oxidation during human fetal development.Growth in Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency.Transport of butyryl-L-carnitine, a potential prodrug, via the carnitine transporter OCTN2 and the amino acid transporter ATB(0,+)LC-quadrupole/Orbitrap high-resolution mass spectrometry enables stable isotope-resolved simultaneous quantification and ¹³C-isotopic labeling of acyl-coenzyme A thioesters.The scientific impact of the Structural Genomics Consortium: a protein family and ligand-centered approach to medically-relevant human proteins.Application of prodrugs to inflammatory diseases of the gut.Metabolism as a complex genetic trait, a systems biology approach: implications for inborn errors of metabolism and clinical diseasesCardiac-specific VLCAD deficiency induces dilated cardiomyopathy and cold intolerance.PGC-1 coactivators in cardiac development and diseaseA matter of life, death and diseases: mitochondria from a proteomic perspective.Pathophysiology of fatty acid oxidation disorders and resultant phenotypic variability.How animals move: comparative lessons on animal locomotion.Very long-chain acyl-CoA dehydrogenase (VLCAD-) deficiency-studies on treatment effects and long-term outcomes in mouse models.Short-chain acyl-CoA dehydrogenase deficiency: from gene to cell pathology and possible disease mechanisms.Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine.Thermolabile phenotype of carnitine palmitoyltransferase II variations as a predisposing factor for influenza-associated encephalopathy.Characterization of carnitine and fatty acid metabolism in the long-chain acyl-CoA dehydrogenase-deficient mouse.Accumulation of 3-hydroxy-fatty acids in the culture medium of long-chain L-3-hydroxyacyl CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein-deficient skin fibroblasts: implications for medium chain triglyceride dietary treatment of L
P2860
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P2860
description
2000 nî lūn-bûn
@nan
2000 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年学术文章
@wuu
2000年学术文章
@zh-cn
2000年学术文章
@zh-hans
2000年学术文章
@zh-my
2000年学术文章
@zh-sg
2000年學術文章
@yue
name
Inborn errors of mitochondrial fatty acid oxidation.
@ast
Inborn errors of mitochondrial fatty acid oxidation.
@en
type
label
Inborn errors of mitochondrial fatty acid oxidation.
@ast
Inborn errors of mitochondrial fatty acid oxidation.
@en
prefLabel
Inborn errors of mitochondrial fatty acid oxidation.
@ast
Inborn errors of mitochondrial fatty acid oxidation.
@en
P2093
P2860
P1476
Inborn errors of mitochondrial fatty acid oxidation.
@en
P2093
P2860
P356
10.1080/10408360091174169
P577
2000-02-01T00:00:00Z