Myopathy associated LDB3 mutation causes Z-disc disassembly and protein aggregation through PKCα and TSC2-mTOR downregulation

Autor: Pankaj Pathak, Yotam Blech-Hermoni, Kalpana Subedi, Jessica Mpamugo, Charissa Obeng-Nyarko, Rachel Ohman, Ilda Molloy, Malcolm Kates, Jessica Hale, Stacey Stauffer, Shyam K. Sharan, Ami Mankodi
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Communications Biology, Vol 4, Iss 1, Pp 1-15 (2021)
Druh dokumentu: article
ISSN: 2399-3642
DOI: 10.1038/s42003-021-01864-1
Popis: Pathak et al. identify LDB3, a striated muscle PDZ-LIM protein, as a signaling adaptor in a major mechanosensor assembly through interactions with filamin C, HSPA8, and PKCα. When LDB3 is mutated, PKCα and TSC2-mTOR mediated homeostasis is impaired, leading to protein aggregation myopathy.
Databáze: Directory of Open Access Journals
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