LONP2

Entrez Summary: 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].
UniProt Summary: 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. {ECO:0000255|HAMAP- Rule:MF_03121, ECO:0000269|PubMed:22002062}.

Pfam Domains GO Terms

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Global Fraction of Cell Lines Where Essential: 0/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	0/28
blood	0/28
bone	0/26
breast	0/33
central nervous system	0/56
cervix	0/4
colorectal	0/17
esophagus	0/13
fibroblast	0/1
gastric	0/16
kidney	0/21
liver	0/20
lung	0/75
lymphocyte	0/16
ovary	0/26
pancreas	0/24
peripheral nervous system	0/16
plasma cell	0/15
prostate	0/1
skin	0/24
soft tissue	0/9
thyroid	0/2
upper aerodigestive	0/22
urinary tract	0/29
uterus	0/5

Expression Distribution

Screen	Score
UM0125461 0.74μM R02 exp84	1.83
Diepoxybutane 3μM R07 exp354	2.43

ATP-dependent peptidase activity
protein import into peroxisome matrix
peroxisomal membrane transport
regulation of fatty acid beta-oxidation
protein quality control for misfolded or incompletely synthesized proteins
regulation of fatty acid oxidation
protein transmembrane import into intracellular organelle
intracellular protein transmembrane transport
peroxisomal matrix
regulation of lipid catabolic process
protein transmembrane transport
establishment of protein localization to peroxisome
protein targeting to peroxisome
protein localization to peroxisome
peroxisomal transport
peroxisome organization
peptidase activity
regulation of fatty acid metabolic process
protease binding
peroxisome
protein import
protein processing
serine-type endopeptidase activity
regulation of cellular ketone metabolic process
protein maturation
enzyme binding
protein targeting
regulation of lipid metabolic process
regulation of small molecule metabolic process
establishment of protein localization to organelle
proteolysis involved in cellular protein catabolic process
cellular protein catabolic process
protein catabolic process
protein localization to organelle
regulation of cellular catabolic process
cellular macromolecule catabolic process
response to organic cyclic compound
intracellular protein transport
regulation of catabolic process
macromolecule catabolic process