A Biochemical Abnormality in Cerebrotendinous Xanthomatosis IMPAIRMENT OF BILE ACID BIOSYNTHESIS ASSOCIATED WITH INCOMPLETE DEGRADATION OF THE CHOLESTEROL SIDE CHAIN

Bile acid production in cerebrotendinous xanthomatosis (CTX) is subnormal, yet the activity of cholesterol 7α-hydroxylase, the rate-determining enzyme of bile acid synthesis, is elevated. To explain this discrepancy, bile acid precursors were sought in bile and feces of three CTX subjects. Over 10%...

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Bibliografiska uppgifter

I publikationen:	J Clin Invest
Huvudupphov:	Setoguchi, T., Salen, Gerald, Tint, G. S., Mosbach, E. H.
Materialtyp:	Artigo
Språk:	Inglês
Utgiven:	American Society for Clinical Investigation 1974
Ämnen:	Articles
Länkar:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC302628/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/4825231/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI107688
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