CPLM-006668

Genbank Nucleotide ID

Lon protease homolog, mitochondrial

Protein Synonyms/Alias

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

Homo sapiens (Human)

Position	Peptide	Type	References
393	KIKQTHRKYLLQEQL	ubiquitination	[1]
579	KIIQCLKKTKTENPL	ubiquitination	[1]

[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]

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. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein.

DOMAIN 124 368 Lon.
NP_BIND 523 530 ATP (By similarity).
ACT_SITE 855 855 By similarity.
ACT_SITE 898 898 By similarity.

3D-structure; ATP-binding; Complete proteome; DNA-binding; Hydrolase; Mitochondrion; Nucleotide-binding; Polymorphism; Protease; Reference proteome; Serine protease; Transit peptide.

UniProt (SWISSPROT/TrEMBL); GenBank; EMBL

GO:0042645; C:mitochondrial nucleoid; IDA:BHF-UCL.
GO:0043531; F:ADP binding; IDA:UniProtKB.
GO:0005524; F:ATP binding; IDA:UniProtKB.
GO:0004176; F:ATP-dependent peptidase activity; IDA:UniProtKB.
GO:0051880; F:G-quadruplex DNA binding; IDA:UniProtKB.
GO:0070362; F:mitochondrial heavy strand promoter anti-sense binding; IDA:UniProtKB.
GO:0070361; F:mitochondrial light strand promoter anti-sense binding; IDA:UniProtKB.
GO:0043565; F:sequence-specific DNA binding; IDA:UniProtKB.
GO:0004252; F:serine-type endopeptidase activity; IBA:RefGenome.
GO:0003697; F:single-stranded DNA binding; IBA:RefGenome.
GO:0003727; F:single-stranded RNA binding; IDA:UniProtKB.
GO:0007568; P:aging; IEA:Compara.
GO:0034599; P:cellular response to oxidative stress; IDA:UniProtKB.
GO:0051131; P:chaperone-mediated protein complex assembly; IEA:HAMAP.
GO:0006515; P:misfolded or incompletely synthesized protein catabolic process; IBA:RefGenome.
GO:0032042; P:mitochondrial DNA metabolic process; NAS:UniProtKB.
GO:0070407; P:oxidation-dependent protein catabolic process; IMP:UniProtKB.
GO:0051260; P:protein homooligomerization; IDA:UniProtKB.
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; IEP:UniProtKB.

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.

PF00004; AAA
PF02190; LON
PF05362; Lon_C

SM00382; AAA
SM00464; LON