3.4.24.B18: m-AAA protease
This is an abbreviated version!
For detailed information about m-AAA protease, go to the full flat file.
Word Map on EC 3.4.24.B18
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3.4.24.B18
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paraplegia
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hereditary
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ataxia
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spinocerebellar
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atpases
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oma1
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proteostasis
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cristae
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dynamin-like
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homo-oligomeric
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paraparesis
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spastin
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hetero-oligomeric
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inner-membrane
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medicine
- 3.4.24.B18
- paraplegia
- hereditary
- ataxia
- spinocerebellar
- atpases
- oma1
-
proteostasis
-
cristae
-
dynamin-like
-
homo-oligomeric
- paraparesis
- spastin
-
hetero-oligomeric
-
inner-membrane
- medicine
Reaction
proteolytic degradation of proteins =
Synonyms
AAA protease, AFG3-like protein 2, Afg3l1, AFG3L2, ClpX, lon protease, m-AAA protease, m-AAA+ protease, M41.003, mitochondrial AAA protease, mitochondrial AAA+ protein, mitochondrial ATPase associated with various activities protease, mitochondrial mAAA protease, mitochondrial respiratory chain complexes assembly protein RCA1, paraplegin, SCRG_02514, SPG7, TAT-binding homolog 12, Yme1L, Yta10, Yta12, YTA12p
ECTree
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Localization
Localization on EC 3.4.24.B18 - m-AAA protease
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both subunit types span the membrane twice, integral, active at the matrix side of the inner membrane
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both subunit types span the membrane twice, integral, active at the matrix side of the inner membrane
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inner side, exposes catalytic sites to the mitochondrial matrix
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integral, active at the matrix side of the inner membrane
the catalytic domain is located in the mitochondrial matrix
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the m-AAA protease is a conserved hetero-oligomeric complex in the inner membrane of mitochondria. m-AAA protease is preferentially localized in the inner boundary membrane compared to the cristae membrane
human mitochondria possess two m-AAA protease isoenzymes: a hetero-oligomeric complex, composed of paraplegin and AFG3L2 (Afg3 like 2), and a homo-oligomeric AFG3L2 complex. Genetic interaction between the m-AAA isoenzymes and suggest that different neuronal populations have variable thresholds of susceptibility to reduced levels of the m-AAA protease
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active on the matrix side of the inner membrane