Tchem LONP1 Lon protease homolog, mitochondrial

Protein Summary

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 (PubMed:12198491, PubMed:15870080, PubMed:17420247, PubMed:8248235). Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not (PubMed:17579211, PubMed:28377575). 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 also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]

Uniprot Accession IDs

• P36776
• B3KPH8
• D6W635
• E5KMH8
• F5GZ27
• P36777
• Q8N8K8
• Q9UQ95

Gene Name

• LONP1

Ensembl ID

• ENST00000360614
• ENSP00000353826
• ENSG00000196365
• ENST00000540670
• ENSP00000441523
• ENST00000593119
• ENSP00000468541

Symbol

• PRSS15
• LON
• LONP
• PIM1
• hLON
• LonHS
• CODASS
• PRSS15

Knowledge Table

Most Knowledge About	Knowledge Value (0 to 1 scale)
molecular function	1
protein domain	1
interacting protein	0.99
transcription factor binding site profile	0.93
kinase perturbation	0.91

Protein Classes

PANTHER Classes

Protein / ... / Serine Protease / Lon Protease Homolog, Mitochondrial

DTO Classes

Protein / Enzyme / Protease / Serine Protease / Lon Protease Homolog, Mitochondrial

IDG Development Level Summary

Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 429.17   (req: < 5)

Gene RIFs: 69   (req: <= 3)

Antibodies: 254   (req: <= 50)

Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 429.17   (req: >= 5)

Gene RIFs: 69   (req: > 3)

Antibodies: 254   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 23

Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligands: 16

Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 1