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Details on Person AFG3L2 (m-AAA+) is a hexameric zinc metalloprotease that is ...
| Class:Id | Summation:9839661 |
|---|---|
| _displayName | AFG3L2 (m-AAA+) is a hexameric zinc metalloprotease that is ... |
| _timestamp | 2023-11-06 05:35:09 |
| created | [InstanceEdit:9839700] May, Bruce, 2023-07-07 |
| literatureReference | [LiteratureReference:9838619] Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia [LiteratureReference:9852648] Pathogenic variants in the AFG3L2 proteolytic domain cause SCA28 through haploinsufficiency and proteostatic stress-driven OMA1 activation |
| modified | [InstanceEdit:9839806] May, Bruce, 2023-07-10 [InstanceEdit:9840442] May, Bruce, 2023-07-18 [InstanceEdit:9852591] May, Bruce, 2023-11-06 |
| text | AFG3L2 (m-AAA+) is a hexameric zinc metalloprotease that is anchored in the mitochondrial inner membrane and protrudes into the mitochondrial matrix (Koppen et al. 2007, Puchades et al. 2019). AFG3L2 is able to assemble as homohexamers or as heterohexamers with its paralog SPG7 (Paraplegin) (Atorino et al. 2003). Each AFG3L2 subunit has a membrane-proximal ATPase domain that unfolds proteins and a membrane-distal protease domain (Puchades et al. 2019). The substrate protein threads through the ATPase spiral of the AFG3L2 hexamer (Puchades et al. 2019) and is processively translocated and degraded in an ATP-dependent manner. AFG3L2 binds, unfolds, and degrades mitochondrial matrix proteins, including steroidogenic acute regulatory protein (STAR) (Bahat et al. 2014), the ribosomal subunit MRPL32 (Koppen et al. 2007, Ding et al. 2018), and the peripheral inner membrane protein ATP5F1A (Cesnekova et al. 2018). Mutations in AFG3L2 have been linked with development of spinocerebellar ataxia type 28 (SCA28), an autosomal dominant neurodegenerative disease (Tulli et al. 2019). |
| (summation) | [Reaction:9839149] AFG3L2 binds mitochondrial matrix proteins [Homo sapiens] |
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No pathways have been reviewed or authored by AFG3L2 (m-AAA+) is a hexameric zinc metalloprotease that is ... (9839661)
