Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency.

We investigated the peroxisomal beta-oxidation system in liver from a patient with clinical features similar to those in the cerebrohepatorenal (Zellweger) syndrome and with elevated levels in body fluids of very-long-chain fatty acids and intermediates in the biosynthesis of bile acids. The peroxis...

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Detalhes bibliográficos
Publicado no:Proc Natl Acad Sci U S A
Main Authors: Schram, A W, Goldfischer, S, van Roermund, C W, Brouwer-Kelder, E M, Collins, J, Hashimoto, T, Heymans, H S, van den Bosch, H, Schutgens, R B, Tager, J M
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 1987
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC304678/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/2882519/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.84.8.2494
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