Pantethine and cystamine deplete cystine from cystinotic fibroblasts via efflux of cysteamine-cysteine mixed disulfide.
Children suffering from cystinosis, a genetic disease characterized by high levels of lysosomal cystine, are currently being treated with cysteamine to lower the cystine levels in their cells. In fibroblasts from these patients, cysteamine and its disulfide, cystamine, are equally effective in lower...
Gardado en:
| Publicado en: | J Clin Invest |
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| Principais autores: | , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado: |
American Society for Clinical Investigation
1984
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| Assuntos: | |
| Acceso en liña: | https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC370491/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/6746900/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI111436 |
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