Calpain-mediated proteolysis of vimentin filaments is augmented in giant axonal neuropathy fibroblasts exposed to hypotonic stress

Calpain-mediated proteolysis of vimentin filaments is augmented in giant axonal neuropathy fibroblasts exposed to hypotonic stress

Giant Axonal Neuropathy (GAN) is a pediatric neurodegenerative disease caused by loss-of-function mutations in the E3 ubiquitin ligase adaptor gigaxonin, which is encoded by the KLHL16 gene. Gigaxonin regulates the degradation of multiple intermediate filament (IF) proteins, including neurofilaments...

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Detalles Bibliográficos
Autores principales: Cassandra L. Phillips, Dong Fu, Laura E. Herring, Diane Armao, Natasha T. Snider
Formato: Artículo
Lenguaje:English
Publicado: Frontiers Media S.A. 2022-11-01
Colección:Frontiers in Cell and Developmental Biology
Materias:
protease
fragments
protein aggregation
gigaxonin
post-translational modifications
Acceso en línea:https://www.frontiersin.org/articles/10.3389/fcell.2022.1008542/full

Internet

https://www.frontiersin.org/articles/10.3389/fcell.2022.1008542/full

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