Version 5.4
Mus musculus Gene: Lonp1
Summary
InnateDB Gene IDBG-192666.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol Lonp1
Gene Name lon peptidase 1, mitochondrial
Synonyms 1200017E13Rik; LON; Prss15
Species Mus musculus
Ensembl Gene ENSMUSG00000041168
Encoded Proteins
IDBP-192668
lon peptidase 1, mitochondrial
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene does not have any Entrez summary - the following is the summary from its human ortholog ENSG00000196365:
This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]
Gene Information
Type Protein coding
Genomic Location Chromosome 17:56614301-56626903
Strand Reverse strand
Band D
Transcripts
ENSMUST00000047226 ENSMUSP00000041814
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 4 experimentally validated interaction(s) in this database.
They are also associated with 25 interaction(s) predicted by orthology.
Experimentally validated
Total 4 [view]
Protein-Protein 4 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 25 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0000166 nucleotide binding
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:0017111 nucleoside-triphosphatase 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: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: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
Orthologs
Species
Homo sapiens
Bos taurus
Gene ID
Gene Order
ENSG00000196365
View Gene Order
ENSBTAG00000001795
Not yet available
Cross-References
SwissProt Q8CGK3
TrEMBL Q56A16
UniProt Splice Variant
Entrez Gene 74142
UniGene Mm.329136 Mm.456753
RefSeq NM_028782
OMIM
CCDS CCDS28910
HPRD
IMGT
MGI ID MGI:1921392
MGI Symbol Lonp1
EMBL AK004820 AK157474 AK159302 AK160071 AK161983 AY190302 BC092212
GenPept AAH92212 AAN85210 BAB23591 BAE34094 BAE34972 BAE35606 BAE36666
RNA Seq Atlas 74142

If you use InnateDB for your research, please cite the following publication:

Breuer et al., InnateDB: systems biology of innate immunity and beyond - recent updates and continuing curation. Nucl. Acids Res. (2013) 41 (D1) 

InnateDB is being developed jointly by the Brinkman Laboratory (Simon Fraser University, British Columbia, Canada), the Hancock Laboratory (University of British Columbia, Vancouver, British Columbia) and the Lynn EMBL Australia Group (South Australian Health & Medical Research Institute and Flinders University, Adelaide, Australia).

Funding is currently provided by Allergen and EMBL Australia. Previous funding has been provided by Genome Canada, the Foundation for the National Institutes of Health through the Grand Challenges in Global Health initiative and by Teagasc. InnateDB curated interactions are licensed under the Design Science License. All other data is licensed under the terms of the originating database. Contact: innatedb-mail@sfu.ca