Cargando…
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...
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 |
Ejemplares similares
-
Intermediate filament dysregulation and astrocytopathy in the human disease model of KLHL16 mutation in giant axonal neuropathy (GAN)
por: Battaglia, Rachel, et al.
Publicado: (2023) -
Explaining intermediate filament accumulation in giant axonal neuropathy
por: Opal, Puneet, et al.
Publicado: (2013) -
Development of Intrathecal AAV9 Gene Therapy for Giant Axonal Neuropathy
por: Bailey, Rachel M., et al.
Publicado: (2018) -
Advancing the pathologic phenotype of giant axonal neuropathy: early involvement of the ocular lens
por: Armao, Diane, et al.
Publicado: (2019) -
Calpain-mediated proteolysis of tropomodulin isoforms leads to thin filament elongation in dystrophic skeletal muscle
por: Gokhin, David S., et al.
Publicado: (2014)