Version 5.4
Homo sapiens Gene: LONP1
Summary
InnateDB Gene IDBG-20471.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol LONP1
Gene Name lon peptidase 1, mitochondrial
Synonyms hLON; LON; LonHS; LONP; PIM1; PRSS15
Species Homo sapiens
Ensembl Gene ENSG00000196365
Encoded Proteins
IDBP-20475
lon peptidase 1, mitochondrial
IDBP-603228
lon peptidase 1, mitochondrial
IDBP-762446
IDBP-764037
IDBP-766018
IDBP-761091
IDBP-765999
IDBP-763742
IDBP-765277
IDBP-763631
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary 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 19:5691834-5720572
Strand Reverse strand
Band p13.3
Transcripts
ENST00000360614 ENSP00000353826
ENST00000540670 ENSP00000441523
ENST00000585374 ENSP00000465585
ENST00000587365 ENSP00000468114
ENST00000590558 ENSP00000467808
ENST00000589473 ENSP00000468379
ENST00000591321
ENST00000590206 ENSP00000468083
ENST00000590729 ENSP00000465139
ENST00000588589
ENST00000586617 ENSP00000468385
ENST00000587552
ENST00000590511
ENST00000593119 ENSP00000468541
ENST00000590728
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:0030163 protein catabolic process
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
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
ENSMUSG00000041168
View Gene Order
ENSBTAG00000001795
Not yet available
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.350265
RefSeq NM_001276479 NM_001276480 NM_004793
HUGO
OMIM
CCDS CCDS12148 CCDS62507 CCDS62508
HPRD 05689
IMGT
EMBL
GenPept
RNA Seq Atlas

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