Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
about
Phosphomevalonate kinase is a cytosolic protein in humansMutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis.Crystal structure of the (R)-specific enoyl-CoA hydratase from Aeromonas caviae involved in polyhydroxyalkanoate biosynthesisPex11p plays a primary role in medium-chain fatty acid oxidation, a process that affects peroxisome number and size in Saccharomyces cerevisiae.Yeast peroxisomal multifunctional enzyme: (3R)-hydroxyacyl-CoA dehydrogenase domains A and B are required for optimal growth on oleic acid.Novel mutations in the PEX12 gene of patients with a peroxisome biogenesis disorderIdentification of the molecular defect in patients with peroxisomal mosaicism using a novel method involving culturing of cells at 40 degrees C: implications for other inborn errors of metabolismIdentification 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.Adrenoleukodystrophy and related disorders.Mutations in the DBP-deficiency protein HSD17B4 cause ovarian dysgenesis, hearing loss, and ataxia of Perrault Syndrome.Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolismMFE1, a member of the peroxisomal hydroxyacyl coenzyme A dehydrogenase family, affects fatty acid metabolism necessary for morphogenesis in Dictyostelium spp.Heimler Syndrome Is Caused by Hypomorphic Mutations in the Peroxisome-Biogenesis Genes PEX1 and PEX6The important role of biochemical and functional studies in the diagnostics of peroxisomal disordersPeroxisomal abnormalities in the immortalized human hepatocyte (IHH) cell line.Metabolic enzymes dysregulation in heart failure: the prospective therapy.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.A homozygous missense variant in HSD17B4 identified in a consanguineous Chinese Han family with type II Perrault syndrome.Downregulation of estrogen-metabolizing 17 beta-hydroxysteroid dehydrogenase type 2 expression correlates inversely with Ki67 proliferation marker in colon-cancer development.Studies on the metabolic fate of n-3 polyunsaturated fatty acids.A novel type of rhizomelic chondrodysplasia punctata, RCDP5, is caused by loss of the PEX5 long isoform.Experimental evidence that pristanic acid disrupts mitochondrial homeostasis in brain of young rats.Engineering polyhydroxyalkanoate content and monomer composition in the oleaginous yeast Yarrowia lipolytica by modifying the ß-oxidation multifunctional protein
P2860
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P2860
Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
description
1999 nî lūn-bûn
@nan
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
1999年论文
@zh
1999年论文
@zh-cn
name
Peroxisomal bifunctional prote ...... enzymatic and molecular basis.
@en
type
label
Peroxisomal bifunctional prote ...... enzymatic and molecular basis.
@en
prefLabel
Peroxisomal bifunctional prote ...... enzymatic and molecular basis.
@en
P2093
P2860
P356
P1476
Peroxisomal bifunctional prote ...... enzymatic and molecular basis.
@en
P2093
Hashimoto T
Mooijer PA
Wanders RJ
Watkins PA
van Berkel E
van Grunsven EG
P2860
P304
P356
10.1086/302180
P407
P577
1999-01-01T00:00:00Z