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