ATPase Family Gene 3 Like Protein 2 (AFG3L2)

AFG3L2 is the catalytic subunit of the m-AAA protease, an ATP-dependent proteolytic complex of the mitochondrial inner membrane that degrades misfolded proteins and regulates ribosome assembly. AFG3L2 encodes a deduced 797-amino acid protein whose sequence shows 69% similarity to the yeast Afg3 mitochondrial ATPase and 49% identity to paraplegin. AFG3L2 contains an AAA (for ATPase associated with diverse cellular activities) domain of about 190 amino acids with an ATP/GTP-binding site, a zinc-dependent binding domain, and an RNA-binding region. Northern blot analysis revealed that AFG3L2 is expressed ubiquitously as a 3.2-kb transcript in fetal and adult tissues, with greatest expression in heart and skeletal muscle.