| Gene Symbol |
LONP1
|
| Entrez Gene |
9361
|
| Alt Symbol |
CODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
lon peptidase 1, mitochondrial
|
| Other Description |
LONP|hLON ATP-dependent protease|lon protease homolog, mitochondrial|lon protease-like protein|mitochondrial ATP-dependent protease Lon|mitochondrial lon protease-like protein|serine protease 15
|
| Swissprots |
Q8N8K8 P36776 B3KPH8 E5KMH8 P36777 Q9UQ95 D6W635 F5GZ27
|
| Accessions |
AAD24414 ADP90374 ADP90375 ADP90376 ADP90377 ADP90378 ADP90379 ADP90380 ADP90381 ADP90382 ADP90383 ADP90384 ADP90385 ADP90386 ADP90387 ADP90388 ADP90389 ADP90390 ADP90391 ADP90392 ADP90393 ADP90394 ADP90395 ADP90396 ADP90397 ADP90398 ADP90399 ADP90400 ADP90401 ADP90402 ADP90403 ADP90404 ADP90405 ADP90406 ADP90407 ADP90408 ADP90409 ADP90410 ADP90411 ADP90412 ADP90413 EAW69151 EAW69152 EAW69153 EAW69154 P36776 AK056366 BAG51690 AK096433 BAG53290 AK096626 BAC04829 AK127867 BAG54587 AK298531 BAG60732 AK309720 AL096720 BC000235 AAH00235 BC109218 AAI09219 BC109219 BC139726 AAI39727 BI906625 DQ892955 ABM83881 DQ896202 ABM87201 U02389 AAA61616 XM_011528441 XP_011526743 NM_001276479 NP_001263408 NM_001276480 NP_001263409 NM_004793 NP_004784 NR_076392
|
| 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:0000255|HAMAP- Rule:MF_03120, ECO:0000269|PubMed:12198491, ECO:0000269|PubMed:15870080, ECO:0000269|PubMed:17420247, ECO:0000269|PubMed:8248235}.
|
| Subcellular Location |
Mitochondrion matrix {ECO:0000255|HAMAP- Rule:MF_03120, ECO:0000269|PubMed:7961901}.
|
| Tissue Specificity |
Duodenum, heart, lung and liver, but not thymus.
|