Entity Details

Primary name LONM_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP36776
EntryNameLONM_HUMAN
FullNameLon protease homolog, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length959
SequenceStatuscomplete
DateCreated1994-06-01
DateModified2021-06-02

Ontological Relatives

GenesLONP1

GO terms

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GOName
GO:0000002 mitochondrial genome maintenance
GO:0001018 mitochondrial promoter sequence-specific DNA binding
GO:0001666 response to hypoxia
GO:0003697 single-stranded DNA binding
GO:0003727 single-stranded RNA binding
GO:0004176 ATP-dependent peptidase activity
GO:0004252 serine-type endopeptidase activity
GO:0005524 ATP binding
GO:0005654 nucleoplasm
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005829 cytosol
GO:0006515 protein quality control for misfolded or incompletely synthesized proteins
GO:0007005 mitochondrion organization
GO:0007568 aging
GO:0009725 response to hormone
GO:0010044 response to aluminum ion
GO:0016020 membrane
GO:0016887 ATP hydrolysis activity
GO:0032042 mitochondrial DNA metabolic process
GO:0034599 cellular response to oxidative stress
GO:0042645 mitochondrial nucleoid
GO:0042802 identical protein binding
GO:0043531 ADP binding
GO:0043565 sequence-specific DNA binding
GO:0051131 chaperone-mediated protein complex assembly
GO:0051603 proteolysis involved in cellular protein catabolic process
GO:0051880 G-quadruplex DNA binding
GO:0070182 DNA polymerase binding
GO:0070407 oxidation-dependent protein catabolic process

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR003111 Lon, substrate-binding domainDomainDomain
IPR003593 AAA+ ATPase domainDomainDomain
IPR003959 ATPase, AAA-type, coreDomainDomain
IPR004815 Lon protease, bacterial/eukaryotic-typeFamilyFamily
IPR008268 Peptidase S16, active siteSiteActive site
IPR008269 Peptidase S16, Lon proteolytic domainDomainDomain
IPR014721 Ribosomal protein S5 domain 2-type fold, subgroupFamilyHomologous superfamily
IPR015947 PUA-like superfamilyFamilyHomologous superfamily
IPR020568 Ribosomal protein S5 domain 2-type foldFamilyHomologous superfamily
IPR027065 Lon proteaseFamilyFamily
IPR027417 P-loop containing nucleoside triphosphate hydrolaseFamilyHomologous superfamily
IPR027503 Lon protease homologue, chloroplastic/mitochondrialFamilyFamily

Diseases

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Disease IDSourceNameDescription
600373 OMIMCODAS syndrome (CODASS)A rare syndrome characterized by the combination of cerebral, ocular, dental, auricular, and skeletal features. These include developmental delay, craniofacial anomalies, cataracts, ptosis, median nasal groove, delayed tooth eruption, hearing loss, short stature, delayed epiphyseal ossification, metaphyseal hip dysplasia, and vertebral coronal clefts. The disease is caused by variants affecting the gene represented in this entry.