Lon peptidase 2, peroxisomal

Lon peptidase 2, peroxisomal is a protein that in humans is encoded by the LONP2 gene.

LONP2
Identifiers
Aliases	LONP2, LONP, LONPL, lon peptidase 2, peroxisomal, PLON, PSLON
External IDs	OMIM: 617774; MGI: 1914137; HomoloGene: 12050; GeneCards: LONP2; OMA:LONP2 - orthologs
Gene location (Human) Chr. Chromosome 16 (human) Band 16q12.1 Start 48,244,300 bp End 48,363,122 bp
Gene location (Mouse) Chr. Chromosome 8 (mouse) Band 8|8 C3 Start 87,350,672 bp End 87,450,501 bp
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in right uterine tube right lobe of liver pituitary gland anterior pituitary left lobe of thyroid gland caput epididymis right lobe of thyroid gland left ovary tibia right ovary Top expressed in left lobe of liver brown adipose tissue white adipose tissue cardiac muscle tissue of left ventricle dentate gyrus of hippocampal formation granule cell intercostal muscle granulocyte muscle of thigh interventricular septum right kidney More reference expression data BioGPS n/a
Gene ontology Molecular function nucleotide binding protease binding peptidase activity protein binding enzyme binding serine-type peptidase activity serine-type endopeptidase activity signaling receptor binding hydrolase activity ATP binding ATP-dependent peptidase activity ATPase activity Cellular component cytoplasm membrane peroxisome peroxisomal matrix nucleus cytosol Biological process protein targeting to peroxisome peroxisome organization response to organic cyclic compound proteolysis regulation of fatty acid beta-oxidation protein catabolic process protein processing protein quality control for misfolded or incompletely synthesized proteins protein import into peroxisome matrix Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 83752 66887 Ensembl ENSG00000102910 ENSMUSG00000047866 UniProt Q86WA8 Q9DBN5 RefSeq (mRNA) NM_001300948 NM_031490 NM_001348078 NM_001168591 NM_025827 RefSeq (protein) NP_001287877 NP_113678 NP_001335007 NP_001162063 NP_080103 Location (UCSC) Chr 16: 48.24 – 48.36 Mb Chr 8: 87.35 – 87.45 Mb PubMed search
Wikidata
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Function

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].

Further reading

• Ondrovicová G, Liu T, Singh K, Tian B, Li H, Gakh O, Perecko D, Janata J, Granot Z, Orly J, Kutejová E, Suzuki CK (2005). "Cleavage site selection within a folded substrate by the ATP-dependent lon protease". J. Biol. Chem. 280 (26): 25103–10. doi:10.1074/jbc.M502796200. PMID 15870080.

This article incorporates text from the United States National Library of Medicine, which is in the public domain.