Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency
about
Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis.The molecular biology, biochemistry, and physiology of human steroidogenesis and its disordersPeroxisomal multifunctional enzyme type 2 from the fruitfly: dehydrogenase and hydratase act as separate entities, as revealed by structure and kineticsYeast peroxisomal multifunctional enzyme: (3R)-hydroxyacyl-CoA dehydrogenase domains A and B are required for optimal growth on oleic acid.Identification of a substrate-binding site in a peroxisomal beta-oxidation enzyme by photoaffinity labeling with a novel palmitoyl derivativeThe role of alpha-methylacyl-CoA racemase in bile acid synthesisIn Silico Functional Annotation of Genomic Variation.Expression and localization of estrogenic type 12 17beta-hydroxysteroid dehydrogenase in the cynomolgus monkeyQuality control: from molecules to organelles.In mouse alpha -methylacyl-CoA racemase, the same gene product is simultaneously located in mitochondria and peroxisomes.The bifunctional protein TtFARAT from Tetrahymena thermophila catalyzes the formation of both precursors required to initiate ether lipid biosynthesis.Importance of Gly-13 for the coenzyme binding of human UDP-glucose dehydrogenase.Plasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls.On the molecular basis of D-bifunctional protein deficiency type III.MFE1, a member of the peroxisomal hydroxyacyl coenzyme A dehydrogenase family, affects fatty acid metabolism necessary for morphogenesis in Dictyostelium spp.Mutations in SRD5B1 (AKR1D1), the gene encoding delta(4)-3-oxosteroid 5beta-reductase, in hepatitis and liver failure in infancy.PEX11 promotes peroxisome division independently of peroxisome metabolismMechanisms of disease: Inborn errors of bile acid synthesis.SILEC: a protocol for generating and using isotopically labeled coenzyme A mass spectrometry standardsDevelopmental roles of D-bifunctional protein-A zebrafish model of peroxisome dysfunction.A structurally conserved water molecule in Rossmann dinucleotide-binding domains.Metabolic enzymes dysregulation in heart failure: the prospective therapy.Identification of mosquito sterol carrier protein-2 inhibitors.A novel HPLC-based method to diagnose peroxisomal D-bifunctional protein enoyl-CoA hydratase deficiency.Reinvestigation of peroxisomal 3-ketoacyl-CoA thiolase deficiency: identification of the true defect at the level of d-bifunctional protein.Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.Structure-based phylogenetic analysis of short-chain alcohol dehydrogenases and reclassification of the 17beta-hydroxysteroid dehydrogenase family.Branched chain fatty acids induce nitric oxide-dependent apoptosis in vascular smooth muscle cells.Studies on the metabolic fate of n-3 polyunsaturated fatty acids.Prioritization of candidate disease genes for metabolic syndrome by computational analysis of its defining phenotypes.Identification of the peroxisomal beta-oxidation enzymes involved in the degradation of long-chain dicarboxylic acids.Loss of an Androgen-Inactivating and Isoform-Specific HSD17B4 Splice Form Enables Emergence of Castration-Resistant Prostate Cancer.Expanding the genotypic spectrum of Perrault syndrome.Quaternary structure of human, Drosophila melanogaster and Caenorhabditis elegans MFE-2 in solution from synchrotron small-angle X-ray scattering.Metabolic etiologies in West syndrome.
P2860
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P2860
Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency
description
1998 nî lūn-bûn
@nan
1998 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
1998 թվականի մարտին հրատարակված գիտական հոդված
@hy
1998年の論文
@ja
1998年論文
@yue
1998年論文
@zh-hant
1998年論文
@zh-hk
1998年論文
@zh-mo
1998年論文
@zh-tw
1998年论文
@wuu
name
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@ast
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en-gb
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@nl
type
label
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@ast
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en-gb
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@nl
prefLabel
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@ast
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en-gb
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@nl
P2093
P2860
P356
P1476
Peroxisomal D-hydroxyacyl-CoA ...... ifunctional protein deficiency
@en
P2093
D A Cuebas
E G van Grunsven
E van Berkel
J B de Klerk
P T Clayton
R J Wanders
P2860
P304
P356
10.1073/PNAS.95.5.2128
P407
P577
1998-03-03T00:00:00Z