A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
about
Two novel mutations in the sterol 27-hydroxylase gene causing cerebrotendinous xanthomatosisFamilial giant cell hepatitis associated with synthesis of 3 beta, 7 alpha-dihydroxy-and 3 beta,7 alpha, 12 alpha-trihydroxy-5-cholenoic acidsBiosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acidDefective peroxisomal cleavage of the C27-steroid side chain in the cerebro-hepato-renal syndrome of Zellweger.Demonstration of 26-hydroxylation of C27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosisBile acid synthesis in man. In vivo activity of the 25-hydroxylation pathway.In vivo and vitro studies on formation of bile acids in patients with Zellweger syndrome. Evidence that peroxisomes are of importance in the normal biosynthesis of both cholic and chenodeoxycholic acid.Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study.Analysis of bioactive oxysterols in newborn mouse brain by LC/MS.Cholic acid biosynthesis: the enzymatic defect in cerebrotendinous xanthomatosisOn the future of "omics": lipidomics.Role of liver peroxisomes in bile acid formation: inborn error of C27-steroid side chain cleavage in peroxisome deficiency (Zellweger syndrome).Kinetics of liver microsomal cholecalciferol 25-hydroxylase in vitamin D-depleted and -repleated rats.Biosynthesis of chenodeoxycholic acid in man: stereospecific side-chain hydroxylations of 5beta-cholestane-3alpha,7alpha-diol.Side chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis.Bioconversion of 3beta-hydroxy-5-cholenoic acid into chenodeoxycholic acid by rat brain enzyme systems.Prospective treatment of cerebrotendinous xanthomatosis with cholic acid therapy.
P2860
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P2860
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
description
1976 nî lūn-bûn
@nan
1976 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1976 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1976年の論文
@ja
1976年論文
@yue
1976年論文
@zh-hant
1976年論文
@zh-hk
1976年論文
@zh-mo
1976年論文
@zh-tw
1976年论文
@wuu
name
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@ast
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@en
type
label
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@ast
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@en
prefLabel
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@ast
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@en
P2093
P2860
P356
P1476
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.
@en
P2093
Mosbach EH
P2860
P304
P356
10.1172/JCI108366
P407
P577
1976-04-01T00:00:00Z