LONP1
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].
| Name | OMIM ID | Ensembl ID | HGNC ID | PHARMGKB ID | Map Location |
|---|---|---|---|---|---|
| lon peptidase 1, mitochondrial | MIM:605490 | Ensembl:ENSG00000196365 | HGNC:HGNC:9479 | PA162394145 | 19p13.3 |
GO terms in LONP1
| Term Type | Evidence Type | GO Term ID | GO Des. |
|---|---|---|---|
| CC | IDA | GO:0005634 | nucleus |
| CC | IDA | GO:0005654 | nucleoplasm |
| CC | IDA | GO:0005739 | mitochondrion |
| CC | IBA | GO:0005759 | mitochondrial matrix |
| CC | IMP | GO:0005759 | mitochondrial matrix |
| CC | IDA | GO:0005829 | cytosol |
| CC | HDA | GO:0016020 | membrane |
| CC | IDA | GO:0042645 | mitochondrial nucleoid |
| BP | NAS | GO:0000002 | mitochondrial genome maintenance |
| BP | IEP | GO:0001666 | response to hypoxia |
| BP | IBA | GO:0006515 | protein quality control for misfolded or incompletely synthesized proteins |
| BP | IBA | GO:0007005 | mitochondrion organization |
| BP | IMP | GO:0007005 | mitochondrion organization |
| BP | IEA | GO:0007568 | aging |
| BP | IEA | GO:0009725 | response to hormone |
| BP | IEA | GO:0010044 | response to aluminum ion |
| BP | NAS | GO:0032042 | mitochondrial DNA metabolic process |
| BP | IC | GO:0034599 | cellular response to oxidative stress |
| BP | IDA | GO:0034599 | cellular response to oxidative stress |
| BP | IEA | GO:0034622 | cellular protein-containing complex assembly |
| BP | IBA | GO:0051131 | chaperone-mediated protein complex assembly |
| BP | IDA | GO:0051260 | protein homooligomerization |
| BP | IDA | GO:0051603 | proteolysis involved in cellular protein catabolic process |
| BP | IMP | GO:0070407 | oxidation-dependent protein catabolic process |
| MF | IDA | GO:0001018 | mitochondrial promoter sequence-specific DNA binding |
| MF | IDA | GO:0001018 | mitochondrial promoter sequence-specific DNA binding |
| MF | IBA | GO:0003697 | single-stranded DNA binding |
| MF | IEA | GO:0003697 | single-stranded DNA binding |
| MF | IDA | GO:0003727 | single-stranded RNA binding |
| MF | IBA | GO:0004176 | ATP-dependent peptidase activity |
| MF | IDA | GO:0004176 | ATP-dependent peptidase activity |
| MF | IEA | GO:0004252 | serine-type endopeptidase activity |
| MF | IPI | GO:0005515 | protein binding |
| MF | IDA | GO:0005524 | ATP binding |
| MF | IDA | GO:0043531 | ADP binding |
| MF | IDA | GO:0043565 | sequence-specific DNA binding |
| MF | IDA | GO:0051880 | G-quadruplex DNA binding |
| MF | IPI | GO:0070182 | DNA polymerase binding |
Gene expression in normal tissue: LONP1
Gene-model tissue-cancer distribution: Bubble Plot
Gene-drug pathway distribution
Pathways in LONP1
| Database | Pathway ID | Pathway Des. |
|---|
Gene-Drug: Aster Plot
| Drug ID | Drug Name | Model Num. |
|---|---|---|
| iGMDRD105 | Triptolide | 3 |
| iGMDRD289 | Parthenolide | 1 |
| iGMDRD318 | PAC-1 | 1 |
| iGMDRD945 | ML214 | 6 |
| iGMDRD210 | AC55649 | 3 |
| iGMDRD409 | Saracatinib | 1 |
| iGMDRD577 | BIX01294 | 2 |
Gene in drug-gene network: Network Plot
Gene-drug targets distribution
Gene Structure: PDB
Models in LONP1
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