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Defective peroxisomal cleavage of the C27-steroid side chain in the cerebro-hepato-renal syndrome of Zellweger.

Based on in vitro work with rat liver, we recently suggested that the peroxisomal fraction is most important for the oxidation of 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid (THCA) into cholic acid. The cerebro-hepato-renal syndrome of Zellweger is a fatal recessive autosomal diso...

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Detalhes bibliográficos
Main Authors: Kase, B F, Björkhem, I, Hågå, P, Pedersen, J I
Formato: Artigo
Idioma:Inglês
Publicado em: 1985
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC423512/
https://ncbi.nlm.nih.gov/pubmed/3973012
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