Background：

In human， peroxisomes function primarily to catalyze fatty acid beta-oxidation and， as a by-product， produce hydrogen peroxide and superoxide. The protein encoded by this gene is an ATP-dependent protease that likely plays a role in maintaining overall peroxisome homeostasis as well as proteolytically degrading peroxisomal proteins damaged by oxidation. The protein has an N-terminal Lon N substrate recognition domain， an ATPase domain， a proteolytic domain， and， in some isoforms， a C-terminal peroxisome targeting sequence. Alternative splicing results in multiple transcript variants encoding distinct isoforms.