Ontology highlight
ABSTRACT:
SUBMITTER: Kondadi AK
PROVIDER: S-EPMC4193934 | biostudies-literature | 2014 May
REPOSITORIES: biostudies-literature
Kondadi Arun Kumar AK Wang Shuaiyu S Montagner Sara S Kladt Nikolay N Korwitz Anne A Martinelli Paola P Herholz David D Baker Michael J MJ Schauss Astrid C AC Langer Thomas T Rugarli Elena I EI
The EMBO journal 20140328 9
The m-AAA protease subunit AFG₃L₂ is involved in degradation and processing of substrates in the inner mitochondrial membrane. Mutations in AFG₃L₂ are associated with spinocerebellar ataxia SCA28 in humans and impair axonal development and neuronal survival in mice. The loss of AFG₃L₂ causes fragmentation of the mitochondrial network. However, the pathogenic mechanism of neurodegeneration in the absence of AFG₃L₂ is still unclear. Here, we show that depletion of AFG₃L₂ leads to a specific defect ...[more]