Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria
about
Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidationcDNA cloning and amino acid sequence of human mitochondrial delta 3 delta 2-enoyl-CoA isomerase: comparison of the human enzyme with its rat counterpart, mitochondrial short-chain isomeraseSystems virology identifies a mitochondrial fatty acid oxidation enzyme, dodecenoyl coenzyme A delta isomerase, required for hepatitis C virus replication and likely pathogenesisObservations regarding retinopathy in mitochondrial trifunctional protein deficienciesCombined enzyme defect of mitochondrial fatty acid oxidationLong-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: a molecular and biochemical reviewEstrogen receptor alpha interacts with mitochondrial protein HADHB and affects beta-oxidation activityCommon missense mutation G1528C in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Characterization and expression of the mutant protein, mutation analysis on genomic DNA and chromosomal localization of the mitochondrial trifunctional proteinHyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretionDisorders of phospholipid metabolism: an emerging class of mitochondrial disease due to defects in nuclear genesThe complexity of cardiolipin in health and diseaseElimination of the unnecessary: intra- and extracellular signaling by anionic phospholipidsMitochondrial Trifunctional Protein Defects: Clinical Implications and Therapeutic ApproachesInborn errors of mitochondrial fatty acid oxidation.Two alpha subunit donor splice site mutations cause human trifunctional protein deficiencyRetinal dystrophy in long chain 3-hydroxy-acyl-coA dehydrogenase deficiency.Prevalence of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency in Estonia.The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results.Structural basis for channelling mechanism of a fatty acid beta-oxidation multienzyme complexMitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients.Organic acidurias and related abnormalities.Genes for the human mitochondrial trifunctional protein alpha- and beta-subunits are divergently transcribed from a common promoter region.Mammalian mitochondrial beta-oxidation.Peroxisomal beta-oxidation and polyunsaturated fatty acids.Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunitsA 13C isotopomer n.m.r. method for monitoring incomplete beta-oxidation of fatty acids in intact tissue.Intramitochondrial control of the oxidation of hexadecanoate in skeletal muscle. A study of the acyl-CoA esters which accumulate during rat skeletal-muscle mitochondrial beta-oxidation of [U-14C]hexadecanoate and [U-14C]hexadecanoyl-carnitineMedium chain 3-ketoacyl-coenzyme A thiolase deficiency: a new disorder of mitochondrial fatty acid beta-oxidation.Acute fatty liver of pregnancy: an update on pathogenesis and clinical implications.Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.A new type of a multifunctional beta-oxidation enzyme in euglena.Pathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation.trans-Hexadec-2-enoyl-CoA+H2O<=>(S)-3-Hydroxyhexadecanoyl-CoA(S)-3-Hydroxyhexadecanoyl-CoA+NAD<=>3-Oxopalmitoyl-CoA+NADH+H3-Oxopalmitoyl-CoA+CoA-SH<=>myristoyl-CoAtrans-Tetradec-2-enoyl-CoA+H2O<=>(S)-3-Hydroxytetradecanoyl-CoA(S)-3-Hydroxytetradecanoyl-CoA+NAD<=>3-Oxotetradecanoyl-CoA+NADH+H3-Oxotetradecanoyl-CoA+CoA-SH<=>Lauroyl-CoA3-Oxododecanoyl-CoA+CoA-SH<=>Decanoyl-CoA3-Oxodecanoyl-CoA+CoA-SH<=>Octanoyl-CoA
P2860
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P2860
Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria
description
1992 nî lūn-bûn
@nan
1992 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
1992 թվականի մարտին հրատարակված գիտական հոդված
@hy
1992年の論文
@ja
1992年論文
@yue
1992年論文
@zh-hant
1992年論文
@zh-hk
1992年論文
@zh-mo
1992年論文
@zh-tw
1992年论文
@wuu
name
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@ast
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en-gb
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@nl
type
label
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@ast
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en-gb
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@nl
prefLabel
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@ast
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en-gb
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@nl
P2093
P921
P1476
Human liver long-chain 3-hydro ...... idation enzyme of mitochondria
@en
P2093
B Middleton
K Carpenter
R J Pollitt
P356
10.1016/0006-291X(92)90501-B
P407
P577
1992-03-16T00:00:00Z