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Protection of Cystinotic Mice by Kidney-Specific Megalin Ablation Supports an Endocytosis-Based Mechanism for Nephropathic Cystinosis Progression
BACKGROUND: Deletions or inactivating mutations of the cystinosin gene CTNS lead to cystine accumulation and crystals at acidic pH in patients with nephropathic cystinosis, a rare lysosomal storage disease and the main cause of hereditary renal Fanconi syndrome. Early use of oral cysteamine to preve...
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| Veröffentlicht in: | J Am Soc Nephrol |
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| Hauptverfasser: | , , , , , , , , , , , , |
| Format: | Artigo |
| Sprache: | Inglês |
| Veröffentlicht: |
American Society of Nephrology
2019
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| Schlagworte: | |
| Online Zugang: | https://ncbi.nlm.nih.gov/pmc/articles/PMC6830792/ https://ncbi.nlm.nih.gov/pubmed/31548351 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1681/ASN.2019040371 |
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