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Abnormal intermediate filament organization alters mitochondrial motility in giant axonal neuropathy fibroblasts

Giant axonal neuropathy (GAN) is a rare disease caused by mutations in the GAN gene, which encodes gigaxonin, an E3 ligase adapter that targets intermediate filament (IF) proteins for degradation in numerous cell types, including neurons and fibroblasts. The cellular hallmark of GAN pathology is the...

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Detalles Bibliográficos
Autores principales: Lowery, Jason, Jain, Nikhil, Kuczmarski, Edward R., Mahammad, Saleemulla, Goldman, Anne, Gelfand, Vladimir I., Opal, Puneet, Goldman, Robert D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The American Society for Cell Biology 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4750921/
https://www.ncbi.nlm.nih.gov/pubmed/26700320
http://dx.doi.org/10.1091/mbc.E15-09-0627