Mitochondrial inner membrane m-AAA protease component; mediates degradation of misfolded or unassembled proteins; also required for correct assembly of mitochondrial enzyme complexes; overexpression of human AFG3L2 complements respiratory defect of yeast afg3 yta12 double null mutation, but overexpression of disease-associated AFG3L2 variants does not; expression of both human SPG7 (paraplegin) and AFG3L2 complements yeast yta12 afg3 double mutation [Source:SGD;Acc:S000004695]
RCA1
Chromosome XIII: 445,609-448,086 reverse strand.
R64-1-1:BK006946.2
This gene has 1 transcript (splice variant), 50 orthologues, 2 paralogues and is a member of 2 Ensembl protein families.