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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: Phillips, Cassandra L., Fu, Dong, Herring, Laura E., Armao, Diane, Snider, Natasha T.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9664965/
https://www.ncbi.nlm.nih.gov/pubmed/36393840
http://dx.doi.org/10.3389/fcell.2022.1008542