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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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Gorde:
Xehetasun bibliografikoak
Egile Nagusiak: Johnson, Jennifer L., Napolitano, Gennaro, Monfregola, Jlenia, Rocca, Celine J., Cherqui, Stephanie, Catz, Sergio D.
Formatua: Artigo
Hizkuntza:Inglês
Argitaratua: American Society for Microbiology 2013
Gaiak:
Sarrera elektronikoa: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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