Recombinant Human Lon protease homolog 2, peroxisomal (LONP2), Truncated

Uniprot Id

Q86WA8

Target Species

Human

Target Name

LONP2

Target Full Name

Lon protease homolog 2, peroxisomal

Target Function

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. May indirectly regulate peroxisomal fatty acid beta-oxidation through degradation of the self-processed forms of TYSND1.

Target Subcellular Location

Peroxisome matrix.

Target Protein Families

Peptidase S16 family

Target Tissue Specificity

Widely expressed, with high levels in the liver, kidney and pancreas.

Target Synonyms

Lon peptidase 2, peroxisomal; Lon protease 2; Lon protease homolog 2; Lon protease homolog 2 peroxisomal; Lon protease like protein 2; Lon protease-like protein 2; LONP; lonp2; LONP2_HUMAN; LONPL; MGC4840; peroxisomal; Peroxisomal Lon protease; Peroxisomal Lon protease homolog 2; Peroxisomal LON protease like

Target 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.