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Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders

Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C(27)-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form prim...

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Detalles Bibliográficos
Autores principales: Heubi, James E., Setchell, Kenneth D.R., Bove, Kevin E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6062720/
https://www.ncbi.nlm.nih.gov/pubmed/30057520
http://dx.doi.org/10.1159/000490095