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Drosophila VCP/p97 Mediates Dynein-Dependent Retrograde Mitochondrial Motility in Axons.


ABSTRACT: Valosin-containing protein (VCP), also called p97, is an evolutionarily conserved and ubiquitously expressed ATPase with diverse cellular functions. Dominant mutations in VCP are found in a late-onset multisystem degenerative proteinopathy. The neurological manifestations of the disorder include frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). In these patients, long motor neuron axons could be particularly susceptible to defects in axonal transport. However, whether VCP has a physiological function in maintaining axonal transport and whether this role is impaired by disease-causing mutations remains elusive. Here, by employing live-imaging methods in Drosophila larval axons and performing genetic interaction experiments, we discover that VCP regulates the axonal tran

SUBMITTER: Gonzalez AE 

PROVIDER: S-EPMC7186335 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature