Afg3l2 - AFG3-like AAA ATPase 2

Predicted to enable metalloendopeptidase activity. Involved in protein autoprocessing. Acts upstream of or within several processes, including mitochondrion organization; nervous system development; and righting reflex. Located in mitochondrial inner membrane. Part of m-AAA complex. Is expressed in brain. Used to study spinocerebellar ataxia type 28. Human ortholog(s) of this gene implicated in optic atrophy 12; spastic ataxia; spastic ataxia 5; and spinocerebellar ataxia type 28. Orthologous to human AFG3L2 (AFG3 like matrix AAA peptidase subunit 2). [provided by Alliance of Genome Resources, Apr 2022]