Disorders of mitochondrial fatty acyl-CoA beta-oxidation
about
2-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 managementAcute fatty liver of pregnancyA general introduction to the biochemistry of mitochondrial fatty acid β-oxidationMitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studiesGeneral mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnoverLong-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: a molecular and biochemical reviewMitochondrial long chain fatty acid beta-oxidation in man and mouseCarnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout miceA phytol-enriched diet induces changes in fatty acid metabolism in mice both via PPARalpha-dependent and -independent pathwaysLack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden deathDynamic simulations on the mitochondrial fatty acid beta-oxidation network.A novel tandem mass spectrometry method for rapid confirmation of medium- and very long-chain acyl-CoA dehydrogenase deficiency in newborns.Women have higher protein content of beta-oxidation enzymes in skeletal muscle than men.Inborn errors of energy metabolism associated with myopathies.Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans.Prenatal diagnosis of disorders of fatty acid transport and mitochondrial oxidation.Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse modelsFatty Acid oxidation disorder with secondary mitochondrial energy production defect: a case reportThe diverse roles of flavin coenzymes--nature's most versatile thespians.Compartmentalized acyl-CoA metabolism in skeletal muscle regulates systemic glucose homeostasisPlasma fatty acids as diagnostic markers in autistic patients from Saudi Arabia.Recombinant adeno-associated virus-mediated gene delivery of long chain acyl coenzyme A dehydrogenase (LCAD) into LCAD-deficient miceIntramuscular triacylglycerol and insulin resistance: guilty as charged or wrongly accused?Activation of human mitochondrial pantothenate kinase 2 by palmitoylcarnitine.Altered Monoamine and Acylcarnitine Metabolites in HIV-Positive and HIV-Negative Subjects With Depression.Reduced mitochondrial mass and function add to age-related susceptibility toward diet-induced fatty liver in C57BL/6J mice.Fatty acid oxidation is a dominant bioenergetic pathway in prostate cancer.Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapyUnique plasma metabolomic signatures of individuals with inherited disorders of long-chain fatty acid oxidation.Acute Fatty Liver of Pregnancy: A Retrospective Analysis of 56 CasesHepatic Fatty Acid Oxidation Restrains Systemic Catabolism during Starvation.Fatty Acid oxidation disorders in a chinese population in taiwan.Low expression of long-chain acyl-CoA dehydrogenase in human skeletal muscleThe enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results.Glycine conjugation: importance in metabolism, the role of glycine N-acyltransferase, and factors that influence interindividual variation.Pathophysiology of fatty acid oxidation disorders and resultant phenotypic variability.Fluoromethylated derivatives of carnitine biosynthesis intermediates--synthesis and applications.Very long-chain acyl-CoA dehydrogenase (VLCAD-) deficiency-studies on treatment effects and long-term outcomes in mouse models.Earlier diagnosis and strict diets improve the survival rate and clinical course of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
P2860
Q24532034-038806FF-02FB-487B-AB2A-DE525B2AE437Q24616340-C2EDC662-17EF-4D70-BB51-11A587E05882Q24649161-F725B6F0-FF1C-4ABF-B378-FF1D440BA1BCQ26699142-319F1074-94F9-429B-9A08-43CB516C8B85Q28085600-B5196BCE-10ED-4DAE-8E5D-7AE673FA7112Q28186172-2A2C0760-16B1-46A4-AA52-1CB652542376Q28214659-5143DEAC-33BB-4991-BA14-FE832C786EBDQ28246062-6873D0F4-B088-4226-8422-C5D7CDC01737Q28288565-F1D94DC5-2B65-48C2-AF87-00C101E22B8DQ28302844-ABFF1B1B-5618-4291-89E4-97BDC292D628Q28505245-3DE8916C-590F-4047-8ACE-F30CA003E85BQ33397846-874252E6-C88C-427B-8975-0F6CCACFFB18Q33489532-ECFA1DF1-D533-4239-AF07-A3F24CBAD542Q33654084-CC2AC24A-B1C9-4EC5-BB84-B56B9DB3D95BQ33879587-9E8D5FC0-FFD9-429E-B75B-4977077B2908Q34025017-A66A3F42-59EF-4AB1-A925-1E192210FDB0Q34141683-31FBA53C-70EF-4423-908B-EBB52A6F0CCCQ34163820-283F631A-D387-45BE-A88D-16CA5575C250Q34246828-93957407-B3C7-49D7-9225-8416476997D4Q34639688-71FDA4CA-6B75-40C5-A6F4-0D4991F7388DQ34762456-624802E5-32BC-45E4-82CF-151C76DB2CEDQ35023635-09FCFFA1-BD19-46ED-8354-40DEF2D554B3Q35055915-B30DAEF3-C331-44C2-840D-90A34B179E4BQ35596985-DF5EAC22-3910-44C4-9420-811B684C6DD0Q35616394-E4CBECE0-E9B0-4B51-B39C-8EE59F6086CFQ36036109-8F8ECE23-4BA3-4D06-BC29-C9D50F6101A1Q36150744-EB57B24A-D4F0-4C41-A4FB-517EF2CF600AQ36472442-396A957A-29A8-4CC0-A0BF-020FF64FB6B3Q36512079-D9E92B24-2F21-46E5-932D-EAD721258AD3Q36854069-9C1A3825-5617-4767-AC1E-E2645BC931AAQ36930151-DCFB446B-BE22-45F8-89F7-99EFF24F4781Q37052937-9F0E8772-BEFB-4E8E-860C-74E74F47DFEDQ37127631-DDE73681-7041-43DF-B624-83718E6FEBC4Q37682615-7F13C954-D296-45A7-A546-9F4EEDBB64FAQ37758547-92201C1C-F643-414A-822C-CDF3604C74B0Q38104736-16BA30A5-DC89-4AA3-908D-7DD39C355EFFQ38106893-7CBCB377-96D0-43B6-AEE4-43AF2573DD9CQ39045890-3F186FCD-0B60-4559-9985-DFEC68EAC21EQ39155672-6AD8131A-9660-4553-BD05-99479FA4F753Q40203013-A4D66884-0FD0-41AE-9F3B-554402026F55
P2860
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
description
1999 nî lūn-bûn
@nan
1999 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի հունիսին հրատարակված գիտական հոդված
@hy
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
name
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@ast
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@en
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@nl
type
label
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@ast
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@en
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@nl
prefLabel
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@ast
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@en
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@nl
P2093
P3181
P356
P1476
Disorders of mitochondrial fatty acyl-CoA beta-oxidation
@en
P2093
A H van Gennip
F A Wijburg
M E den Boer
R J Wanders
P2888
P304
P3181
P356
10.1023/A:1005504223140
P407
P577
1999-06-01T00:00:00Z
P6179
1048078728