Fibrocystin/Polyductin releases a C-terminal fragment that translocates into mitochondria and suppresses cystogenesis

Fibrocystin/Polyductin (FPC), encoded by PKHD1, is associated with autosomal recessive polycystic kidney disease (ARPKD), yet its precise role in cystogenesis remains unclear. Here we show that FPC undergoes complex proteolytic processing in developing kidneys, generating three soluble C-terminal fr...

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Detalles Bibliográficos
Autores principales: Walker, Rebecca V, Yao, Qin, Xu, Hangxue, Maranto, Anthony, Swaney, Kristen F, Ramachandran, Sreekumar, Li, Rong, Cassina, Laura, Polster, Brian M, Outeda, Patricia, Boletta, Alessandra, Watnick, Terry, Qian, Feng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2023
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10579373/
https://www.ncbi.nlm.nih.gov/pubmed/37845212
http://dx.doi.org/10.1038/s41467-023-42196-4

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