LONP1 - Gene info - The Human Protein Atlas

Gene name

LONP1 (HGNC Symbol)

Synonyms

hLON, LonHS, PIM1, PRSS15

Description

Lon peptidase 1, mitochondrial (HGNC Symbol)

Entrez gene summary

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]

Chromosome

Cytoband

p13.3

Chromosome location (bp)

5691834 - 5720572

Protein evidence

Evidence at protein level (all genes)

Ensembl

ENSG00000196365 (version 78.38)

Entrez gene

9361

UniProt

P36776 (UniProt - Evidence at protein level)

Splice variant

Ensembl

UniProt

Protein class

Gene ontology

Length
(aa)

Molecular mass
(kDa)

Signal peptide
(predicted)

Transmembrane regions
(predicted)

LONP1-001

ENSP00000353826
ENST00000360614

P36776
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P36776 [Direct mapping]
Lon protease homolog, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03120}
K7EKE6 [Target identity:88%; Query identity:100%]
Lon protease homolog, mitochondrial {ECO:0000256|HAMAP-Rule:MF_03120}

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Enzymes
Peptidases
Serine-type peptidases
Predicted intracellular proteins
Mitochondrial proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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GO:0000002 [mitochondrial genome maintenance]
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:0005515 [protein binding]
GO:0005524 [ATP binding]
GO:0005737 [cytoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006200 [ATP catabolic process]
GO:0006281 [DNA repair]
GO:0006310 [DNA recombination]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0007005 [mitochondrion organization]
GO:0007568 [aging]
GO:0009378 [four-way junction helicase activity]
GO:0009725 [response to hormone]
GO:0010044 [response to aluminum ion]
GO:0016020 [membrane]
GO:0016887 [ATPase activity]
GO:0032042 [mitochondrial DNA metabolic process]
GO:0034599 [cellular response to oxidative stress]
GO:0042645 [mitochondrial nucleoid]
GO:0043531 [ADP binding]
GO:0043565 [sequence-specific DNA binding]
GO:0043623 [cellular protein complex assembly]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0051260 [protein homooligomerization]
GO:0051603 [proteolysis involved in cellular protein catabolic process]
GO:0051880 [G-quadruplex DNA binding]
GO:0070182 [DNA polymerase binding]
GO:0070361 [mitochondrial light strand promoter anti-sense binding]
GO:0070362 [mitochondrial heavy strand promoter anti-sense binding]
GO:0070407 [oxidation-dependent protein catabolic process]
GO:0090296 [regulation of mitochondrial DNA replication]

959

106.5

LONP1-002

ENSP00000441523
ENST00000540670

P36776
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P36776 [Direct mapping]
Lon protease homolog, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03120}

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Enzymes
Peptidases
Serine-type peptidases
Predicted intracellular proteins
Mitochondrial proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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GO:0000002 [mitochondrial genome maintenance]
GO:0001666 [response to hypoxia]
GO:0003727 [single-stranded RNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005515 [protein binding]
GO:0005524 [ATP binding]
GO:0005737 [cytoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006281 [DNA repair]
GO:0006310 [DNA recombination]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0007005 [mitochondrion organization]
GO:0009378 [four-way junction helicase activity]
GO:0016020 [membrane]
GO:0016887 [ATPase activity]
GO:0032042 [mitochondrial DNA metabolic process]
GO:0034599 [cellular response to oxidative stress]
GO:0042645 [mitochondrial nucleoid]
GO:0043531 [ADP binding]
GO:0043565 [sequence-specific DNA binding]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0051260 [protein homooligomerization]
GO:0051603 [proteolysis involved in cellular protein catabolic process]
GO:0051880 [G-quadruplex DNA binding]
GO:0070182 [DNA polymerase binding]
GO:0070361 [mitochondrial light strand promoter anti-sense binding]
GO:0070362 [mitochondrial heavy strand promoter anti-sense binding]
GO:0070407 [oxidation-dependent protein catabolic process]
GO:0090296 [regulation of mitochondrial DNA replication]

763

85.6

LONP1-003

ENSP00000465585
ENST00000585374

K7EKE6
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K7EKE6 [Target identity:100%; Query identity:100%]
Lon protease homolog, mitochondrial {ECO:0000256|HAMAP-Rule:MF_03120}

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Predicted intracellular proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005737 [cytoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006281 [DNA repair]
GO:0006310 [DNA recombination]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0009378 [four-way junction helicase activity]
GO:0016887 [ATPase activity]
GO:0034599 [cellular response to oxidative stress]
GO:0043565 [sequence-specific DNA binding]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0070407 [oxidation-dependent protein catabolic process]
GO:0090296 [regulation of mitochondrial DNA replication]

845

95.2

LONP1-005

ENSP00000468541
ENST00000593119

P36776
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P36776 [Direct mapping]
Lon protease homolog, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03120}
K7EKE6 [Target identity:94%; Query identity:100%]
Lon protease homolog, mitochondrial {ECO:0000256|HAMAP-Rule:MF_03120}

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Enzymes
Peptidases
Serine-type peptidases
Predicted intracellular proteins
Mitochondrial proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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895

100.4

LONP1-007

ENSP00000465139
ENST00000590729

K7EJE8
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K7EJE8 [Target identity:100%; Query identity:100%]
Lon protease homolog, mitochondrial {ECO:0000256|HAMAP-Rule:MF_03120}

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MEMSAT-SVM predicted membrane proteins
Predicted intracellular proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005737 [cytoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006200 [ATP catabolic process]
GO:0006281 [DNA repair]
GO:0006310 [DNA recombination]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0009378 [four-way junction helicase activity]
GO:0016887 [ATPase activity]
GO:0034599 [cellular response to oxidative stress]
GO:0043565 [sequence-specific DNA binding]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0070407 [oxidation-dependent protein catabolic process]
GO:0090296 [regulation of mitochondrial DNA replication]

829

93.3

LONP1-014

ENSP00000468083
ENST00000590206

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Predicted intracellular proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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GO:0005524 [ATP binding]
GO:0006281 [DNA repair]
GO:0006310 [DNA recombination]
GO:0009378 [four-way junction helicase activity]
GO:0016887 [ATPase activity]

242

27.5

LONP1-015

ENSP00000468114
ENST00000587365

K7ER56
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K7ER56 [Target identity:100%; Query identity:100%]
Lon protease homolog, mitochondrial {ECO:0000313|Ensembl:ENSP00000468114}

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Predicted intracellular proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

Show » « Hide

GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0006200 [ATP catabolic process]
GO:0006508 [proteolysis]
GO:0030163 [protein catabolic process]

210

22.6

LONP1-016

ENSP00000468379
ENST00000589473

K7ERR6
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K7ERR6 [Target identity:100%; Query identity:100%]
Lon protease homolog, mitochondrial {ECO:0000313|Ensembl:ENSP00000468379}

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Predicted intracellular proteins
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)

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264

27.8

The Human Protein Atlas project is funded by the Knut & Alice Wallenberg foundation.