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. Diseases associated with LONP2 include Zellweger Syndrome. Among its related pathways are Chaperonin-mediated protein folding and Metabolism of proteins. 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 Summary 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.