LONP1 - Lon protease homolog, mitochondrial - human protein (Function)
 
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LONP1 »  Lon protease homolog, mitochondrial   [ EC 3.4.21.- ]
 
Protein also known as:  Mitochondrial ATP-dependent protease Lon.
Gene name:  LONP1
Family name: Peptidase S16
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 39 2
GENE REF ISO

Function

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Overview 
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.  
4
  • CuratedUniProtKB
GO molecular function 
ADP bindingdefinition[GO:0043531]  
1
  • IDAUniProtKB
ATP bindingdefinition[GO:0005524]  
1
  • IDAUniProtKB
ATP-dependent peptidase activitydefinition[GO:0004176]  
2
  • IDAUniProtKB
DNA polymerase bindingdefinition[GO:0070182]  
1
  • IPIUniProtKB
G-quadruplex DNA bindingdefinition[GO:0051880]  
1
  • IDAUniProtKB
Mitochondrial heavy strand promoter anti-sense bindingdefinition[GO:0070362]  
1
  • IDAUniProtKB
Not  Mitochondrial heavy strand promoter sense bindingdefinition[GO:0070364]  
1
  • IDAUniProtKB
Mitochondrial light strand promoter anti-sense bindingdefinition[GO:0070361]  
1
  • IDAUniProtKB
Not  Mitochondrial light strand promoter sense bindingdefinition[GO:0070363]  
1
  • IDAUniProtKB
Protein bindingdefinition[GO:0005515]  
2
  • IPIIntAct
  • IPIUniProtKB
Sequence-specific DNA bindingdefinition[GO:0043565]  
1
  • IDAUniProtKB
Serine-type endopeptidase activitydefinition[GO:0004252]  
  • IEAUniProtKB Annot
Single-stranded DNA bindingdefinition[GO:0003697] silver  
  • IEAOrtholog Compara
Single-stranded RNA bindingdefinition[GO:0003727]  
1
  • IDAUniProtKB
GO biological process 
Agingdefinition[GO:0007568] silver  
  • IEAOrtholog Compara
Cellular response to oxidative stressdefinition[GO:0034599]  
2
  • IDAUniProtKB
Chaperone-mediated protein complex assemblydefinition[GO:0051131]  
  • IEAUniProtKB Annot
Misfolded or incompletely synthesized protein catabolic processdefinition[GO:0006515]  
  • IEAUniProtKB Annot
Mitochondrial DNA metabolic processdefinition[GO:0032042]  
1
  • NASUniProtKB
Mitochondrial genome maintenancedefinition[GO:0000002]  
1
  • NASUniProtKB
Mitochondrion organizationdefinition[GO:0007005]  
1
  • IMPUniProtKB
Oxidation-dependent protein catabolic processdefinition[GO:0070407]  
1
  • IMPUniProtKB
Protein homooligomerizationdefinition[GO:0051260]  
1
  • IDAUniProtKB
Proteolysis involved in cellular protein catabolic processdefinition[GO:0051603]  
2
  • IDAUniProtKB
Regulation of mitochondrial DNA replicationdefinition[GO:0090296]  
  • IEAUniProtKB Annot
Response to aluminum iondefinition[GO:0010044] silver  
  • IEAOrtholog Compara
Response to hormonedefinition[GO:0009725] silver  
  • IEAOrtholog Compara
Response to hypoxiadefinition[GO:0001666]  
1
  • IEPUniProtKB
Enzymatic activity 
 
More information is available from:
 

Keywords

Molecular function 
Hydrolase  definition   [KW-0378]
Protease  definition   [KW-0645]
Serine protease  definition   [KW-0720]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GeneWiki: LONP1
GenomeRNAi: 9361
PRO: PR:P36776