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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...
| Autores principales: | , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
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S. Karger AG
2018
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| 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 |