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 C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primar...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2018-06-01
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| Series: | Case Reports in Gastroenterology |
| Subjects: | |
| Online Access: | https://www.karger.com/Article/FullText/490095 |