LONP1 report

I. Expression across cell types

II. Expression across tissues

III. Associated gene sets

GO_0035694Biological processmitochondrial protein catabolic process
GO_0032042Biological processmitochondrial DNA metabolic process
GO_0046627Biological processnegative regulation of insulin receptor signaling pathway
GO_0070407Biological processoxidation-dependent protein catabolic process
GO_0051131Biological processchaperone-mediated protein complex assembly
GO_0050730Biological processregulation of peptidyl-tyrosine phosphorylation
GO_0034599Biological processcellular response to oxidative stress
GO_0007005Biological processmitochondrion organization
GO_0001666Biological processresponse to hypoxia
GO_0030163Biological processprotein catabolic process
GO_0051603Biological processproteolysis involved in protein catabolic process
GO_0000002Biological processmitochondrial genome maintenance
GO_0006515Biological processprotein quality control for misfolded or incompletely synthesized proteins
GO_0042645Cellular componentmitochondrial nucleoid
GO_0016020Cellular componentmembrane
GO_0005739Cellular componentmitochondrion
GO_0005654Cellular componentnucleoplasm
GO_0005829Cellular componentcytosol
GO_0005759Cellular componentmitochondrial matrix
GO_0043531Molecular functionADP binding
GO_0004176Molecular functionATP-dependent peptidase activity
GO_0003727Molecular functionsingle-stranded RNA binding
GO_0043565Molecular functionsequence-specific DNA binding
GO_0070182Molecular functionDNA polymerase binding
GO_0003697Molecular functionsingle-stranded DNA binding
GO_0042731Molecular functionPH domain binding
GO_0042802Molecular functionidentical protein binding
GO_0043560Molecular functioninsulin receptor substrate binding
GO_0051880Molecular functionG-quadruplex DNA binding
GO_0001018Molecular functionmitochondrial promoter sequence-specific DNA binding
GO_0005524Molecular functionATP binding
GO_0005515Molecular functionprotein binding
GO_0004252Molecular functionserine-type endopeptidase activity
GO_0016887Molecular functionATP hydrolysis activity

IV. Literature review

[source]
Gene nameLONP1
Protein nameLon peptidase 1, mitochondrial
Lon protease homolog (EC 3.4.21.-)
Lon protease homolog, mitochondrial (EC 3.4.21.53) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)
LONP1 protein
Lon protease homolog, mitochondrial (EC 3.4.21.53) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)
SynonymsPRSS15
DescriptionFUNCTION: 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. .

FUNCTION: 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. .

FUNCTION: 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 . Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, DELE1, helicase Twinkle (TWNK) and the large ribosomal subunit protein MRPL32/bL32m . MRPL32/bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not . May also have a chaperone function in the assembly of inner membrane protein complexes (By similarity). 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 . .

FUNCTION: 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. .

FUNCTION: 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. .

FUNCTION: 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. .

FUNCTION: 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. .

AccessionsB4DPX0
ENST00000585374.5
ENST00000590729.5
ENST00000587365.1
K7ERS1
K7ER27
B3KU28
K7ERR6
ENST00000540670.6 [P36776-3]
ENST00000589473.1
A4VCH6
K7EQF8
K7ER56
ENST00000360614.8 [P36776-1]
ENST00000590206.1
K7EKE6
E5KMI6
K7EJE8
ENST00000590558.5
ENST00000593119.5 [P36776-2]
P36776
Q2VPA0
ENST00000586617.1
B3KXS5