InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: LONP1

Summary

InnateDB Protein

IDBP-20475.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

LONP1

Protein Name

lon peptidase 1, mitochondrial

Synonyms

hLON; LON; LonHS; LONP; PIM1; PRSS15;

Species

Homo sapiens

Ensembl Protein

ENSP00000353826

InnateDB Gene

IDBG-20471 (LONP1)

Protein Structure

UniProt Annotation

Function

ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single- stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site- specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein. {ECO:0000255HAMAP- Rule:MF_03120, ECO:0000269PubMed:12198491, ECO:0000269PubMed:15870080, ECO:0000269PubMed:17420247, ECO:0000269PubMed:8248235}.

Subcellular Localization

Mitochondrion matrix {ECO:0000255HAMAP- Rule:MF_03120, ECO:0000269PubMed:7961901}.

Disease Associations

Tissue Specificity

Duodenum, heart, lung and liver, but not thymus.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 36 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.

Experimentally validated
Total	36 [view]
Protein-Protein	35 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	1 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003697	single-stranded DNA binding
GO:0003727	single-stranded RNA binding
GO:0004176	ATP-dependent peptidase activity
GO:0004252	serine-type endopeptidase activity
GO:0005515	protein binding
GO:0005524	ATP binding
GO:0009378	four-way junction helicase activity
GO:0016887	ATPase activity
GO:0043531	ADP binding
GO:0043565	sequence-specific DNA binding
GO:0051880	G-quadruplex DNA binding
GO:0070182	DNA polymerase binding
GO:0070361	mitochondrial light strand promoter anti-sense binding
GO:0070362	mitochondrial heavy strand promoter anti-sense binding

Biological Process

GO:0000002	mitochondrial genome maintenance
GO:0001666	response to hypoxia
GO:0006200	ATP catabolic process
GO:0006281	DNA repair
GO:0006310	DNA recombination
GO:0006508	proteolysis
GO:0006515	misfolded or incompletely synthesized protein catabolic process
GO:0007005	mitochondrion organization
GO:0007568	aging
GO:0009725	response to hormone
GO:0010044	response to aluminum ion
GO:0032042	mitochondrial DNA metabolic process
GO:0034599	cellular response to oxidative stress
GO:0043623	cellular protein complex assembly
GO:0051131	chaperone-mediated protein complex assembly
GO:0051260	protein homooligomerization
GO:0051603	proteolysis involved in cellular protein catabolic process
GO:0070407	oxidation-dependent protein catabolic process
GO:0090296	regulation of mitochondrial DNA replication

Cellular Component

GO:0005737	cytoplasm
GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix
GO:0016020	membrane
GO:0042645	mitochondrial nucleoid

Protein Structure and Domains

PDB ID

InterPro

IPR003111 Peptidase S16, lon N-terminal
IPR003593 AAA+ ATPase domain
IPR003959 ATPase, AAA-type, core
IPR004815 Lon protease, bacterial/eukaryotic-type
IPR008269 Peptidase S16, Lon C-terminal
IPR008824 DNA helicase, Holliday junction RuvB type, N-terminal
IPR011704 ATPase, dynein-related, AAA domain
IPR013153 PrkA AAA domain
IPR015947 PUA-like domain
IPR020568 Ribosomal protein S5 domain 2-type fold
IPR027417 P-loop containing nucleoside triphosphate hydrolase

PFAM