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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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Detalles Bibliográficos
Autores principales:	Heubi, James E., Bishop, Warren P.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	S. Karger AG 2018
Materias:	Single Case
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276768/ https://www.ncbi.nlm.nih.gov/pubmed/30519152 http://dx.doi.org/10.1159/000494555

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