Identifiers Symbols LONP1; LON; LONP; LonHS; PIM1; PRSS15; hLON External IDs OMIM: 605490 MGI: 1921392 HomoloGene: 3521 GeneCards: LONP1 Gene

Gene Ontology
Molecular function single-stranded DNA binding
single-stranded RNA binding
ATP-dependent peptidase activity
serine-type endopeptidase activity
protein binding
ATP binding
ADP binding
sequence-specific DNA binding
G-quadruplex DNA binding
DNA polymerase binding
mitochondrial light strand promoter anti-sense binding
mitochondrial heavy strand promoter anti-sense binding
mitochondrial light strand promoter sense binding
mitochondrial heavy strand promoter sense binding
Cellular component cytoplasm
mitochondrial matrix
mitochondrial nucleoid
Biological process mitochondrial genome maintenance
response to hypoxia
misfolded or incompletely synthesized protein catabolic process
mitochondrion organization
mitochondrial DNA metabolic process
cellular response to oxidative stress
protein homooligomerization
proteolysis involved in cellular protein catabolic process
oxidation-dependent protein catabolic process
Sources: Amigo / QuickGO
RNA expression pattern More reference expression data Orthologs Species Human Mouse Entrez 9361 74142 Ensembl ENSG00000196365 ENSMUSG00000041168 UniProt P36776 Q8CGK3 RefSeq (mRNA) NM_004793 NM_028782 RefSeq (protein) NP_004784 NP_083058 Location (UCSC) Chr 19:
5.69 – 5.72 Mb Chr 17:
56.61 – 56.63 Mb PubMed search

Lon protease homolog, mitochondrial is an enzyme that in humans is encoded by the LONP1 gene.

This gene encodes a mitochondrial matrix protein in the Lon family of ATP-dependent proteases. A similar E. coli protein regulates gene expression by targeting specific regulatory proteins for degradation. This protein binds a specific sequence in the light and heavy chain promoters of the mitochondrial genome which are involved in regulation of DNA replication and transcription.