A novel pathway for biosynthesis of cholestanol with 7 alpha-hydroxylated C27-steroids as intermediates, and its importance for the accumulation of cholestanol in cerebrotendinous xanthomatosis
about
Cerebrotendinous xanthomatosis: a comprehensive review of pathogenesis, clinical manifestations, diagnosis, and managementOn the mechanism of accumulation of cholestanol in the brain of mice with a disruption of sterol 27-hydroxylase.Retinal and nonocular abnormalities in Cyp27a1(-/-)Cyp46a1(-/-) mice with dysfunctional metabolism of cholesterol.Identification of bile acid precursors as endogenous ligands for the nuclear xenobiotic pregnane X receptor.Demonstration of 26-hydroxylation of C27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosisProfiling sterols in cerebrotendinous xanthomatosis: utility of Girard derivatization and high resolution exact mass LC-ESI-MS(n) analysisA blood test for cerebrotendinous xanthomatosis with potential for disease detection in newborns.Studies of the mechanism of the increased biosynthesis of cholestanol in cerebrotendinous xanthomatosis. The activity of delta 5-3 beta-hydroxysteroid dehydrogenase.On the mechanism of cerebral accumulation of cholestanol in patients with cerebrotendinous xanthomatosis.Cytochrome P450 27A1 Deficiency and Regional Differences in Brain Sterol Metabolism Cause Preferential Cholestanol Accumulation in the Cerebellum.Thyroid Hormone Status in Sitosterolemia Is Modified by Ezetimibe.Historical aspects of cerebrotendinous xanthomatosis (CTX).
P2860
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P2860
A novel pathway for biosynthesis of cholestanol with 7 alpha-hydroxylated C27-steroids as intermediates, and its importance for the accumulation of cholestanol in cerebrotendinous xanthomatosis
description
1985 nî lūn-bûn
@nan
1985 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
1985 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
1985年の論文
@ja
1985年論文
@yue
1985年論文
@zh-hant
1985年論文
@zh-hk
1985年論文
@zh-mo
1985年論文
@zh-tw
1985年论文
@wuu
name
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@ast
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@en
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@nl
type
label
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@ast
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@en
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@nl
prefLabel
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@ast
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@en
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@nl
P2093
P2860
P356
P1476
A novel pathway for biosynthes ...... cerebrotendinous xanthomatosis
@en
P2093
P2860
P304
P356
10.1172/JCI111719
P407
P577
1985-02-01T00:00:00Z