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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Autors principals: 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
Format: Artigo
Idioma:Inglês
Publicat: 1987
Matèries:
Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC304678/
https://ncbi.nlm.nih.gov/pubmed/2882519
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