UniProt Accession

 LONM_MOUSE; Q8CGK3; Q3TSK9; Q3TVL2; Q3TXE4; Q3TZW3; Q9DBP9 

Genbank Protein ID

 AY190302; AK004820; AK157474; AK159302; AK160071; AK161983 

Genbank Nucleotide ID

 AAN85210.1; BAB23591.1; BAE34094.1; BAE34972.1; BAE35606.1; BAE36666.1 

Protein Name

 Lon protease homolog, mitochondrial 

Protein Synonyms/Alias

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

Gene Name

 Lonp1 

Gene Synonyms/Alias

 Prss15 

Created Date

 July 27, 2013 

Organism

 Mus musculus (Mouse) 

NCBI Taxa ID

 10090 

Lysine Modification

Position	Peptide	Type	References
221	GLEPEAEKQKSRRKL	acetylation	[1]
363	KKEFELSKLQQRLGR	acetylation	[1, 2, 3]
363	KKEFELSKLQQRLGR	succinylation	[3]
375	LGREVEEKIKQTHRK	acetylation	[1]
382	KIKQTHRKYLLQEQL	acetylation	[2]
400	KKELGLEKDDKDAIE	acetylation	[1, 4]
409	DKDAIEEKFRERLRE	acetylation	[1, 4]
707	NLQKQVEKVLRKAAY	acetylation	[1]

Reference

 [1] Calorie restriction and SIRT3 trigger global reprogramming of the mitochondrial protein acetylome.
 Hebert AS, Dittenhafer-Reed KE, Yu W, Bailey DJ, Selen ES, Boersma MD, Carson JJ, Tonelli M, Balloon AJ, Higbee AJ, Westphall MS, Pagliarini DJ, Prolla TA, Assadi-Porter F, Roy S, Denu JM, Coon JJ.
 Mol Cell. 2013 Jan 10;49(1):186-99. [PMID: 23201123]
 [2] Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.
 Rardin MJ, Newman JC, Held JM, Cusack MP, Sorensen DJ, Li B, Schilling B, Mooney SD, Kahn CR, Verdin E, Gibson BW.
 Proc Natl Acad Sci U S A. 2013 Apr 16;110(16):6601-6. [PMID: 23576753]
 [3] SIRT5-Mediated Lysine Desuccinylation Impacts Diverse Metabolic Pathways.
 Park J, Chen Y, Tishkoff DX, Peng C, Tan M, Dai L, Xie Z, Zhang Y, Zwaans BM, Skinner ME, Lombard DB, Zhao Y.
 Mol Cell. 2013 Jun 27;50(6):919-30. [PMID: 23806337]
 [4] Quantification of mitochondrial acetylation dynamics highlights prominent sites of metabolic regulation.
 Still AJ, Floyd BJ, Hebert AS, Bingman CA, Carson JJ, Gunderson DR, Dolan BK, Grimsrud PA, Dittenhafer-Reed KE, Stapleton DS, Keller MP, Westphall MS, Denu JM, Attie AD, Coon JJ, Pagliarini DJ.
 J Biol Chem. 2013 Jul 17;. [PMID: 23864654] 

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 112 357 Lon.
 NP_BIND 512 519 ATP (By similarity).
 ACT_SITE 844 844 By similarity.
 ACT_SITE 887 887 By similarity.  

Keyword

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

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 949 AA 

Protein Sequence

Gene Ontology

 GO:0042645; C:mitochondrial nucleoid; IBA:RefGenome.
 GO:0043531; F:ADP binding; IEA:Compara.
 GO:0005524; F:ATP binding; IBA:RefGenome.
 GO:0004176; F:ATP-dependent peptidase activity; IDA:MGI.
 GO:0051880; F:G-quadruplex DNA binding; IEA:Compara.
 GO:0070361; F:mitochondrial light strand promoter anti-sense binding; IDA:MGI.
 GO:0043565; F:sequence-specific DNA binding; IEA:HAMAP.
 GO:0004252; F:serine-type endopeptidase activity; IBA:RefGenome.
 GO:0003697; F:single-stranded DNA binding; IDA:MGI.
 GO:0003727; F:single-stranded RNA binding; IBA:RefGenome.
 GO:0007568; P:aging; IEA:Compara.
 GO:0034599; P:cellular response to oxidative stress; IBA:RefGenome.
 GO:0051131; P:chaperone-mediated protein complex assembly; IBA:RefGenome.
 GO:0006515; P:misfolded or incompletely synthesized protein catabolic process; IBA:RefGenome.
 GO:0007005; P:mitochondrion organization; IBA:RefGenome.
 GO:0070407; P:oxidation-dependent protein catabolic process; IBA:RefGenome.
 GO:0051260; P:protein homooligomerization; IBA:RefGenome.
 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; IBA:RefGenome. 

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.
 IPR015947; PUA-like_domain.
 IPR020568; Ribosomal_S5_D2-typ_fold.
 IPR014721; Ribosomal_S5_D2-typ_fold_subgr. 

Pfam

 PF00004; AAA
 PF02190; LON
 PF05362; Lon_C 

SMART

 SM00382; AAA
 SM00464; LON 

PROSITE

 PS01046; LON_SER 

PRINTS