UniProt Accession

 F5GZ27_HUMAN; F5GZ27 

Genbank Protein ID

 AC011499 

Genbank Nucleotide ID

  

Protein Name

 Lon protease homolog, mitochondrial 

Protein Synonyms/Alias

 Lon protease-like protein; Mitochondrial ATP-dependent protease Lon; Serine protease 15 

Gene Name

 LONP1 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
197	KIKQTHRKYLLQEQL	ubiquitination	[1]
383	KIIQCLKKTKTENPL	ubiquitination	[1]
552	NLQDFVGKPVFTVER	acetylation	[2]

Reference

 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [2] Proteomic investigations of lysine acetylation identify diverse substrates of mitochondrial deacetylase sirt3.
 Sol EM, Wagner SA, Weinert BT, Kumar A, Kim HS, Deng CX, Choudhary C.
 PLoS One. 2012;7(12):e50545. [PMID: 23236377] 

Functional Description

 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 (By similarity). 

Sequence Annotation

 DOMAIN 7 172 Lon (By similarity).
 NP_BIND 327 334 ATP (By similarity).
 ACT_SITE 659 659 By similarity.
 ACT_SITE 702 702 By similarity.  

Keyword

 ATP-binding; Complete proteome; DNA-binding; Hydrolase; Mitochondrion; Nucleotide-binding; Protease; Reference proteome; Serine protease; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 763 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; IEA:UniProtKB-SubCell.
 GO:0005739; C:mitochondrion; IDA:HPA.
 GO:0005524; F:ATP binding; IEA:HAMAP.
 GO:0004176; F:ATP-dependent peptidase activity; IEA:HAMAP.
 GO:0070361; F:mitochondrial light strand promoter anti-sense binding; IEA:Compara.
 GO:0043565; F:sequence-specific DNA binding; IEA:HAMAP.
 GO:0004252; F:serine-type endopeptidase activity; IEA:HAMAP.
 GO:0003697; F:single-stranded DNA binding; IEA:Compara.
 GO:0007568; P:aging; IEA:Compara.
 GO:0034599; P:cellular response to oxidative stress; IEA:HAMAP.
 GO:0051131; P:chaperone-mediated protein complex assembly; IEA:HAMAP.
 GO:0006515; P:misfolded or incompletely synthesized protein catabolic process; IEA:HAMAP.
 GO:0007005; P:mitochondrion organization; IEA:Compara.
 GO:0070407; P:oxidation-dependent protein catabolic process; IEA:HAMAP.
 GO:0090296; P:regulation of mitochondrial DNA replication; IEA:HAMAP.
 GO:0010044; P:response to aluminum ion; IEA:Compara.
 GO:0009725; P:response to hormone stimulus; IEA:Compara.
 GO:0001666; P:response to hypoxia; IEA:Compara. 

Interpro

 IPR003593; AAA+_ATPase.
 IPR003959; ATPase_AAA_core.
 IPR004815; Lon_bac/euk-typ.
 IPR027065; Lon_Prtase.
 IPR027503; lonm_euk.
 IPR027417; P-loop_NTPase.
 IPR008269; Pept_S16_C.
 IPR003111; Pept_S16_N.
 IPR008268; Peptidase_S16_AS.
 IPR020568; Ribosomal_S5_D2-typ_fold.
 IPR014721; Ribosomal_S5_D2-typ_fold_subgr. 

Pfam

 PF00004; AAA
 PF02190; LON
 PF05362; Lon_C 

SMART

 SM00382; AAA 

PROSITE

 PS01046; LON_SER 

PRINTS