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Long-Term Cholic Acid Treatment in a Patient with Zellweger Spectrum Disorder

Zellweger spectrum disorders (ZSDs) are a subgroup of peroxisomal biogenesis disorders with a generalized defect in peroxisome function. Liver disease in ZSDs has been associated with the lack of peroxisomal β-oxidation of C(27)-bile acid intermediates to form primary C(24)-bile acids, which prevent...

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Detalhes bibliográficos
Publicado no:Case Rep Gastroenterol
Main Authors: Heubi, James E., Bishop, Warren P.
Formato: Artigo
Idioma:Inglês
Publicado em: S. Karger AG 2018
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6276768/
https://ncbi.nlm.nih.gov/pubmed/30519152
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1159/000494555
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