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The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion

Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent potentially lethal monogenic disorder. Mutations in the PKD1 gene, which encodes polycystin-1 (PC1), account for approximately 78% of cases. PC1 is a large 462-kDa protein that undergoes cleavage in its N and C-terminal domai...

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Detalles Bibliográficos
Autores principales: Onuchic, Laura, Padovano, Valeria, Schena, Giorgia, Rajendran, Vanathy, Dong, Ke, Shi, Xiaojian, Pandya, Raj, Rai, Victoria, Gresko, Nikolay P., Ahmed, Omair, Lam, TuKiet T., Wang, Weiwei, Shen, Hongying, Somlo, Stefan, Caplan, Michael J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10063565/
https://www.ncbi.nlm.nih.gov/pubmed/36997516
http://dx.doi.org/10.1038/s41467-023-37449-1