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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Veröffentlicht in:Mol Biol Cell
Hauptverfasser: Lowery, Jason, Jain, Nikhil, Kuczmarski, Edward R., Mahammad, Saleemulla, Goldman, Anne, Gelfand, Vladimir I., Opal, Puneet, Goldman, Robert D.
Format: Artigo
Sprache:Inglês
Veröffentlicht: The American Society for Cell Biology 2016
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Articles
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4750921/
https://ncbi.nlm.nih.gov/pubmed/26700320
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1091/mbc.E15-09-0627
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