Summary

Name AFG3L2
Full Name AFG3-like protein 2
Synonyms Paraplegin-like protein |
Primary ID Q9Y4W6
Links - -
Type protein
Relations 1
Function ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (PubMed:27642048). Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed:22354088).

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Relations

Regulator Mechanism target score ℹ
+ AFG3L2form complex img/form-complex.png binding M-AAA protease complex, AFG3L2-SPG7 variant 0.432
Publications: 1 Organism: Homo Sapiens