Aliases for LONP2 Gene
External Ids for LONP2 Gene
Previous GeneCards Identifiers for LONP2 Gene
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. [provided by RefSeq, Jan 2017]
GeneCards Summary for LONP2 Gene
LONP2 (Lon Peptidase 2, Peroxisomal) is a Protein Coding gene. Among its related pathways are Metabolism of proteins and Chaperonin-mediated protein folding. Gene Ontology (GO) annotations related to this gene include signaling receptor binding and ATPase activity. An important paralog of this gene is LONP1.
UniProtKB/Swiss-Prot for LONP2 Gene
ATP-dependent serine protease that mediates the selective degradation of misfolded and unassembled polypeptides in the peroxisomal matrix. Necessary for type 2 peroxisome targeting signal (PTS2)-containing protein processing and facilitates peroxisome matrix protein import (By similarity). May indirectly regulate peroxisomal fatty acid beta-oxidation through degradation of the self-processed forms of TYSND1.