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LONP1

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GENERAL INFORMATION

Gene name

 LONP1

Gene description

 Lon peptidase 1, mitochondrial

Protein class

 Disease related genes
Enzymes
Potential drug targets
Predicted intracellular proteins

Predicted localization

 Intracellular

Number of transcripts

 8
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HUMAN PROTEIN ATLAS INFORMATION

Summary

 Mainly localized to the mitochondria. In addition localized to the nucleus.

RNA cell category

 Expressed in all

Protein evidence

 Evidence at protein level

Main location

 Localized to the Mitochondria (validated)

Additional location

 In addition localized to the Nucleus (validated)

Single-cell variation

 Single-cell variation in protein expression observed.

DATA RELIABILITY

Reliability score

 Validated based on 2 antibodies.
HPA002034 , HPA002192
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ASSAYS
RNA expression
Human cells

RNA EXPRESSION OVERVIEW
RNA cell line category: Expressed in all
Organ
Origin
Category
Expression
Alphabetical
Cell lines sorted after organ of phenotypic resemblance.

HUMAN CELLS

Summary

 Mainly localized to the mitochondria. In addition localized to the nucleus.

Main location

 Mitochondria (validated)

Additional location

 Nucleus (validated)

 

 

 
Toggle channels
Low
High
G1
S
G2
M
N/A

Thumbnail

Antibody

Cell line

Location

Single-cell variation

Cell cycle dependent variation
HPA002034 A-431 Nucleus
Mitochondria
HPA002034 U-2 OS Nucleus
Mitochondria
Cytosol
HPA002034 U-251 MG Nucleus
Mitochondria
HPA002192 A-431 Nucleoplasm
Mitochondria
HPA002192 U-251 MG Nucleoplasm Spatial
Mitochondria Spatial

GENE INFORMATION

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

 19

Cytoband

 p13.3

Chromosome location (bp)

 5691834 - 5720572

Protein evidence

 Evidence at protein level (all genes)

Ensembl

 ENSG00000196365 (version 83.38)

Entrez gene

 9361

UniProt

 P36776 (UniProt - Evidence at protein level)

neXtProt

 NX_P36776

Antibodypedia

 LONP1 antibodies

PROTEIN BROWSER
LONP1-001
LONP1-002
LONP1-003
LONP1-005
LONP1-007
LONP1-014
LONP1-015
LONP1-016

PROTEIN INFORMATION

Splice variant

UniProt

Protein class

Gene ontology

Length & mass

Signal peptide
(predicted)

Transmembrane regions
(predicted)
LONP1-001
ENSP00000353826
ENST00000360614
P36776 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Enzymes
   Peptidases
   Serine-type peptidases
Predicted intracellular proteins
Disease related genes
Potential drug targets
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)
Show all
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:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0007005 [mitochondrion organization]
GO:0007568 [aging]
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]
Show all
 959 aa
106.5 kDa 		No 0
LONP1-002
ENSP00000441523
ENST00000540670
P36776 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Enzymes
   Peptidases
   Serine-type peptidases
Predicted intracellular proteins
Disease related genes
Potential drug targets
Protein evidence (Ezkurdia et al 2014)
Show all
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:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0007005 [mitochondrion organization]
GO:0016020 [membrane]
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]
Show all
 763 aa
85.6 kDa 		No 0
LONP1-003
ENSP00000465585
ENST00000585374
K7EKE6 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003697 [single-stranded DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005737 [cytoplasm]
GO:0005739 [mitochondrion]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0051131 [chaperone-mediated protein complex assembly]
Show all
 845 aa
95.2 kDa 		No 0
LONP1-005
ENSP00000468541
ENST00000593119
P36776 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Enzymes
   Peptidases
   Serine-type peptidases
Predicted intracellular proteins
Disease related genes
Potential drug targets
Protein evidence (Ezkurdia et al 2014)
Show all
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:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0007005 [mitochondrion organization]
GO:0016020 [membrane]
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]
Show all
 895 aa
100.4 kDa 		No 0
LONP1-007
ENSP00000465139
ENST00000590729
K7EJE8 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
   MEMSAT-SVM predicted membrane proteins
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003697 [single-stranded DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005737 [cytoplasm]
GO:0005739 [mitochondrion]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0051131 [chaperone-mediated protein complex assembly]
Show all
 829 aa
93.3 kDa 		No 0
LONP1-014
ENSP00000468083
ENST00000590206
K7ER27 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003697 [single-stranded DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0051131 [chaperone-mediated protein complex assembly]
Show all
 242 aa
27.5 kDa 		No 0
LONP1-015
ENSP00000468114
ENST00000587365
K7ER56 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003697 [single-stranded DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0051131 [chaperone-mediated protein complex assembly]
Show all
 210 aa
22.6 kDa 		No 0
LONP1-016
ENSP00000468379
ENST00000589473
K7ERR6 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003697 [single-stranded DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0006508 [proteolysis]
GO:0006515 [misfolded or incompletely synthesized protein catabolic process]
GO:0051131 [chaperone-mediated protein complex assembly]
Show all
 264 aa
27.8 kDa 		No 0

Contact

The Project

The Human Protein Atlas

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