Upregulation of the Rab27a-Dependent Trafficking and Secretory Mechanisms Improves Lysosomal Transport, Alleviates Endoplasmic Reticulum Stress, and Reduces Lysosome Overload in Cystinosis

Cystinosis is a lysosomal storage disorder caused by the accumulation of the amino acid cystine due to genetic defects in the CTNS gene, which encodes cystinosin, the lysosomal cystine transporter. Although many cellular dysfunctions have been described in cystinosis, the mechanisms leading to these...

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Autores principales: Johnson, Jennifer L., Napolitano, Gennaro, Monfregola, Jlenia, Rocca, Celine J., Cherqui, Stephanie, Catz, Sergio D.
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Microbiology 2013
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Articles
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3719680/
https://ncbi.nlm.nih.gov/pubmed/23716592
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1128/MCB.00417-13
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