Di- and trihydroxycholestanaemia in twin sisters.

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作者信息

Wanders R J, van Roermund C W, Schelen A, Schutgens R B, Zeman J, Kozich V, Hyanek J, Casteels M, Mannaerts G P

机构信息

Department of Paediatrics, University of Amsterdam, The Netherlands.

出版信息

J Inherit Metab Dis. 1991;14(3):357-60. doi: 10.1007/BF01811702.

DOI:10.1007/BF01811702

PMID:1770791

Abstract

摘要

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Subcellular localization of 3 alpha, 7 alpha-dihydroxy- and 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoyl-coenzyme A ligase(s) in rat liver.

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Deficient oxidation of trihydroxycoprostanic acid in liver homogenates from patients with peroxisomal diseases.

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Subcellular distribution and characteristics of trihydroxycoprostanoyl-CoA synthetase in rat liver.

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Eur J Clin Invest. 1987 Oct;17(5):386-90. doi: 10.1111/j.1365-2362.1987.tb01131.x.

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