• Gene id: 9361

Gene Summary

• Gene Symbol: LONP1   Gene   UCSC   Ensembl
• Aliases: CODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON
• Gene name: lon peptidase 1, mitochondrial
• Alternate names: lon protease homolog, mitochondrial, hLON ATP-dependent protease, lon protease-like protein, mitochondrial ATP-dependent protease Lon, mitochondrial lon protease-like protein, serine protease 15,
• Gene location: 19p13.3 (5720451: 5691833)     Exons: 21     NC_000019.10
• Gene summary(Entrez): This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may a
• OMIM: 607581

Protein Summary

• Protein general information: P36776  
  • Name: Lon protease homolog, mitochondrial (EC 3.4.21.53) (LONHs) (Lon protease like protein) (LONP) (Mitochondrial ATP dependent protease Lon) (Serine protease 15)
  • Length: 959  
  • Mass: 106489
  • Tissue specificity: Duodenum, heart, lung and liver, but not thymus.
• Sequence:

  MAASTGYVRLWGAARCWVLRRPMLAAAGGRVPTAAGAWLLRGQRTCDASPPWALWGRGPAIGGQWRGFWEASSRG
  GGAFSGGEDASEGGAEEGAGGAGGSAGAGEGPVITALTPMTIPDVFPHLPLIAITRNPVFPRFIKIIEVKNKKLV
  ELLRRKVRLAQPYVGVFLKRDDSNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVMGHRRVHISRQLEVEPE
  EPEAENKHKPRRKSKRGKKEAEDELSARHPAELAMEPTPELPAEVLMVEVENVVHEDFQVTEEVKALTAEIVKTI
  RDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVLEETNIPKRLYKALSLLKKEFELSKL
  QQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLKELVVPKHVMDVVDEELSKLGLLDN
  HSSEFNVTRNYLDWLTSIPWGKYSNENLDLARAQAVLEEDHYGMEDVKKRILEFIAVSQLRGSTQGKILCFYGPP
  GVGKTSIARSIARALNREYFRFSVGGMTDVAEIKGHRRTYVGAMPGKIIQCLKKTKTENPLILIDEVDKIGRGYQ
  GDPSSALLELLDPEQNANFLDHYLDVPVDLSKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLV
  PQARALCGLDESKAKLSSDVLTLLIKQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPENLQDFVGKPV
  FTVERMYDVTPPGVVMGLAWTAMGGSTLFVETSLRRPQDKDAKGDKDGSLEVTGQLGEVMKESARIAYTFARAFL
  MQHAPANDYLVTSHIHLHVPEGATPKDGPSAGCTIVTALLSLAMGRPVRQNLAMTGEVSLTGKILPVGGIKEKTI
  AAKRAGVTCIVLPAENKKDFYDLAAFITEGLEVHFVEHYREIFDIAFPDEQAEALAVER

• Structural information:
  • Protein Domains: (124..36-)
    (/note="Lon-N-terminal)
    (/evidence="ECO:0000255|PROSITE-ProRule:PRU01123-)
    (759..94-)
    (/note="Lon-proteolytic)
    (/evidence="ECO:0000255|PROSITE-ProRule:PRU01122"-)
  • Interpro: IPR003593  IPR003959  IPR004815  IPR008269  IPR027065   IPR003111  IPR027503  IPR027417  IPR008268  IPR015947  IPR020568  IPR014721  
  • Prosite: PS51787 PS51786 PS01046
  • PDB: 2X36
  • PDBsum: 2X36
  • STRING: ENSP00000353826
• Other Databases: GeneCards:  LONP1  Malacards:  LONP1

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0007005	mitochondrion organization	IBA	biological process
GO:0051131	chaperone-mediated protein complex assembly	IBA	biological process
GO:0003697	single-stranded DNA binding	IBA	molecular function
GO:0004176	ATP-dependent peptidase activity	IBA	molecular function
GO:0005759	mitochondrial matrix	IBA	cellular component
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	IBA	biological process
GO:0004252	serine-type endopeptidase activity	IEA	molecular function
GO:0006508	proteolysis	IEA	biological process
GO:0004176	ATP-dependent peptidase activity	IEA	molecular function
GO:0005524	ATP binding	IEA	molecular function
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	IEA	biological process
GO:0030163	protein catabolic process	IEA	biological process
GO:0008233	peptidase activity	IEA	molecular function
GO:0016787	hydrolase activity	IEA	molecular function
GO:0006508	proteolysis	IEA	biological process
GO:0003677	DNA binding	IEA	molecular function
GO:0000166	nucleotide binding	IEA	molecular function
GO:0005739	mitochondrion	IEA	cellular component
GO:0008236	serine-type peptidase activity	IEA	molecular function
GO:0005524	ATP binding	IEA	molecular function
GO:0005515	protein binding	IPI	molecular function
GO:0007568	aging	IEA	biological process
GO:0007005	mitochondrion organization	IEA	biological process
GO:0006508	proteolysis	IEA	biological process
GO:0001666	response to hypoxia	IEA	biological process
GO:0006508	proteolysis	IEA	biological process
GO:0034622	cellular protein-containing complex assembly	IEA	biological process
GO:0010044	response to aluminum ion	IEA	biological process
GO:0009725	response to hormone	IEA	biological process
GO:0005759	mitochondrial matrix	IEA	cellular component
GO:0005739	mitochondrion	IEA	cellular component
GO:0004176	ATP-dependent peptidase activity	IEA	molecular function
GO:0016887	ATPase activity	IEA	molecular function
GO:0005739	mitochondrion	IEA	cellular component
GO:0004176	ATP-dependent peptidase activity	IEA	molecular function
GO:0003697	single-stranded DNA binding	IEA	molecular function
GO:0042645	mitochondrial nucleoid	IDA	cellular component
GO:0005759	mitochondrial matrix	IEA	cellular component
GO:0005739	mitochondrion	IDA	cellular component
GO:0005654	nucleoplasm	IDA	cellular component
GO:0005829	cytosol	IDA	cellular component
GO:0004252	serine-type endopeptidase activity	IEA	molecular function
GO:0070407	oxidation-dependent protein catabolic process	IEA	biological process
GO:0043565	sequence-specific DNA binding	IEA	molecular function
GO:0034599	cellular response to oxidative stress	IEA	biological process
GO:0051131	chaperone-mediated protein complex assembly	IEA	biological process
GO:0005759	mitochondrial matrix	IEA	cellular component
GO:0005524	ATP binding	IEA	molecular function
GO:0004176	ATP-dependent peptidase activity	IEA	molecular function
GO:0016887	ATPase activity	IEA	molecular function
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	IEA	biological process
GO:0001018	mitochondrial promoter sequence-specific DNA binding	IDA	molecular function
GO:0051603	proteolysis involved in cellular protein catabolic process	IDA	biological process
GO:0051603	proteolysis involved in cellular protein catabolic process	IDA	biological process
GO:0051880	G-quadruplex DNA binding	IDA	molecular function
GO:0005739	mitochondrion	IDA	cellular component
GO:0005524	ATP binding	IDA	molecular function
GO:0003727	single-stranded RNA binding	IDA	molecular function
GO:0043531	ADP binding	IDA	molecular function
GO:0004176	ATP-dependent peptidase activity	IDA	molecular function
GO:0004176	ATP-dependent peptidase activity	IDA	molecular function
GO:0001018	mitochondrial promoter sequence-specific DNA binding	IDA	NOT|molecular function
GO:0043565	sequence-specific DNA binding	IDA	molecular function
GO:0034599	cellular response to oxidative stress	IDA	biological process
GO:0034599	cellular response to oxidative stress	IC	biological process
GO:0016020	membrane	HDA	cellular component
GO:0032042	mitochondrial DNA metabolic process	NAS	biological process
GO:0001666	response to hypoxia	IEP	biological process
GO:0016887	ATPase activity	IMP	molecular function
GO:0004176	ATP-dependent peptidase activity	IMP	molecular function
GO:0070182	DNA polymerase binding	IPI	molecular function
GO:0005759	mitochondrial matrix	IMP	cellular component
GO:0005515	protein binding	IPI	molecular function
GO:0070407	oxidation-dependent protein catabolic process	IMP	biological process
GO:0007005	mitochondrion organization	IMP	biological process
GO:0042802	identical protein binding	IPI	molecular function
GO:0000002	mitochondrial genome maintenance	NAS	biological process

Diseases

Associated diseases	References
CODAS syndrome	KEGG:H01824
CODAS syndrome	KEGG:H01824
Cryptorchidism	MIK: 28606200
Unexplained infertility	MIK: 25753583

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
28606200	Cryptorchidism			Monozgotic twins (1 control, I cwith cryptorchidism)	Male infertility	MeDIP-Seq
28606200	Cryptorchidism			Monozgotic twins (1 control, I cwith cryptorchidism)	Male infertility	MeDIP-Seq
25753583	Unexplained infertility			46 (17 fertile men, 29 male patients)	Male infertility	Microarray