Protein: P36776 | MoonDB v2.0

UniprotKB AC	UniprotKB ID	Gene name	Full name	Species	Curated set
P36776 (Uniprot)	LONM_HUMAN	LONP1	Lon protease homolog, mitochondrial	human	Yes

Protein Function

Uniprot: 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 i more..

Dissimilar Functions Manual Curation

Function 1: Mitochondrial protease (ATP-dependant mitochondrial protease).

Function 2: 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.

Publications (PMID): 9149530, 17420247

MoonDB Network Modules

Biological Processes

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Module ID (MoonGO)	GO ID (BP)	GO Name
129	GO:0044267	cellular protein metabolic process
129	GO:0007165	signal transduction

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Cellular Components

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Module ID (MoonGO)	GO ID (CC)	GO Name
129	GO:0005829	cytosol
129	GO:0070013	intracellular organelle lumen
293	GO:0005634	nucleus
600	GO:0005634	nucleus
717	GO:0005829	cytosol
717	GO:0043232	intracellular non-membrane-bounded organelle
717	GO:0005634	nucleus

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Protein GO Annotations

Biological Processes

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GO ID (BP)	GO Name	Evidence Code (GO EC)
GO:0000002	mitochondrial genome maintenance	NAS
GO:0001666	response to hypoxia	IEP
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	IBA
GO:0007005	mitochondrion organization	IMP
GO:0007568	aging	IEA
GO:0009725	response to hormone	IEA
GO:0010044	response to aluminum ion	IEA
GO:0032042	mitochondrial DNA metabolic process	NAS
GO:0034599	cellular response to oxidative stress	IDA
GO:0043623	cellular protein complex assembly	IEA

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Cellular Components

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GO ID (CC)	GO Name	Evidence Code (GO EC)
GO:0005634	nucleus	IDA
GO:0005654	nucleoplasm	IDA
GO:0005739	mitochondrion	IDA
GO:0005759	mitochondrial matrix	IMP
GO:0005829	cytosol	IDA
GO:0016020	membrane	IDA
GO:0042645	mitochondrial nucleoid	IDA

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Dissimilar Cellular Components (PrOnto)

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GO ID 1	Component 1	GO ID 2	Component 2	Association Probability (PrOnto)	Interaction Probability (PrOnto)
GO:0005634	nucleus	GO:0016020	membrane	0.00e+00	4.39e-236
GO:0005654	nucleoplasm	GO:0016020	membrane	5.76e-255	1.32e-206

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All Protein Interactions

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Interactor	Also a MoonDB EMF protein
A2MG_HUMAN	Yes: P01023 (MoonDB)
APOE_HUMAN	Yes: P02649 (MoonDB)
CDC37_HUMAN	Yes: Q16543 (MoonDB)
CE164_HUMAN	No
CNTRL_HUMAN	No
CP2C8_HUMAN	No
DCTN1_HUMAN	No
ECSIT_HUMAN	No
FTM_HUMAN	No
IFIT3_HUMAN	No

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Publications Associated

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PMID	Article Title
7961901	Synthesis, processing, and localization of human Lon protease.
8119403	Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases.
8248235	A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease.
9485316	The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner.
12198491	Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism.
14702039	Complete sequencing and characterization of 21,243 full-length human cDNAs.
14739292	DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate.
15057824	The DNA sequence and biology of human chromosome 19.
15489334	The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15870080	Cleavage site selection within a folded substrate by the ATP-dependent lon protease.

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OMIM Diseases Associated

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OMIM ID	Disease Name
600373	CODAS syndrome

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Protein Domains Associated

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Domain Name	Domain ID	Source
AAA	PF00004	Pfam
AAA	SM00382	SMART
AAA+_ATPase	IPR003593	InterPro
ATPase_AAA_core	IPR003959	InterPro
LON	SM00464	SMART
lon	TIGR00763	TIGRFAMs
Lon_bac/euk-typ	IPR004815	InterPro
Lon_C	PF05362	Pfam
LON_N	PS51787	PROSITE
Lon_proteolytic	IPR008269	InterPro

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