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LONP1
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  • LONP1
PROTEIN STRUCTURE
ANTIBODIES
AND
VALIDATION
Protein structures
GENERAL INFORMATIONi

General description of the gene and the encoded protein(s) using information from HGNC and Ensembl, as well as predictions made by the Human Protein Atlas project.

Gene namei

Official gene symbol, which is typically a short form of the gene name, according to HGNC.

LONP1
Synonyms hLON, LonHS, PIM1, PRSS15
Gene descriptioni

Full gene name according to HGNC.

Lon peptidase 1, mitochondrial
Protein classi

Assigned HPA protein class(es) for the encoded protein(s).

Read more
Disease related genes
Enzymes
Human disease related genes
Potential drug targets
Predicted locationi

All transcripts of all genes have been analyzed regarding the location(s) of corresponding protein based on prediction methods for signal peptides and transmembrane regions.

  • Genes with at least one transcript predicted to encode a secreted protein, according to prediction methods or to UniProt location data, have been further annotated and classified with the aim to determine if the corresponding protein(s) are secreted or actually retained in intracellular locations or membrane-attached.

  • Remaining genes, with no transcript predicted to encode a secreted protein, will be assigned the prediction-based location(s).

The annotated location overrules the predicted location, so that a gene encoding a predicted secreted protein that has been annotated as intracellular will have intracellular as the final location.

Read more
Intracellular
Protein evidence Evidence at protein level (all genes)
GENE INFORMATIONi

Gene information from Ensembl and Entrez, as well as links to available gene identifiers are displayed here. Information was retrieved from Ensembl if not indicated otherwise.

Chromosome 19
Cytoband p13.3
Chromosome location (bp) 5691834 - 5720572
Number of transcriptsi

Number of protein-coding transcripts from the gene as defined by Ensembl.

8
Ensembl ENSG00000196365 (version 103.38)
Entrez gene 9361
HGNC HGNC:9479
UniProt P36776 (UniProt - Evidence at protein level)
neXtProt NX_P36776
Antibodypedia LONP1 antibodies


PROTEIN STRUCTUREi

The Structure section provides predicted structures from the Alphafold protein structure database and available experimental structures from Protein Data Bank (PDB).

In the Structure drop-down menu all experimental structures from PDB are available for selection and display. The structures are displayed using the NGL Viewer and can be zoomed-in and rotated either manually or by checking the Autorotate box. The Color scheme can be selected to show the residue index, chain name or confidence score (as B-factors and pLDDT score for experimental and predicted structures, respectively). The positions for available antigen sequences in the structure are shown if Antigens is turned to ON, and the Variants slider can be used to show the positions of clinical and population variants.https://github.com/nglviewer/ngl

Read more

Predicted


Description: Structure prediction from Alphafold project. Structure version 2

# Chains: 1      # Clinical variants: 4      # Population variants: 730

Experimental

Description: P2c-state of wild type human mitochondrial LONP1 protease with bound endogenous substrate protein and in presence of ATP/ADP mix (Electron microscopy)

# Chains: 2      # Clinical variants: 0      # Population variants: 0

Antigens:

Off
On

Variants:

Off
Clinical
Population

Color scheme:

Confidence
Residue index
Chain name

Autorotate:

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On



PROTEIN BROWSERi

The protein browser displays the antigen location on the target protein(s) and the features of the target protein. The tabs at the top of the protein view section can be used to switch between the different splice variants to which an antigen has been mapped.

At the top of the view, the position of the antigen (identified by the corresponding HPA identifier) is shown as a green bar. A yellow triangle on the bar indicates a <100% sequence identity to the protein target.

Below the antigens, the maximum percent sequence identity of the protein to all other proteins from other human genes is displayed, using a sliding window of 10 aa residues (HsID 10) or 50 aa residues (HsID 50). The region with the lowest possible identity is always selected for antigen design, with a maximum identity of 60% allowed for designing a single-target antigen (read more).

The curve in blue displays the predicted antigenicity i.e. the tendency for different regions of the protein to generate an immune response, with peak regions being predicted to be more antigenic.The curve shows average values based on a sliding window approach using an in-house propensity scale. (read more).

If a signal peptide is predicted by a majority of the signal peptide predictors SPOCTOPUS, SignalP 4.0, and Phobius (turquoise) and/or transmembrane regions (orange) are predicted by MDM, these are displayed.

Low complexity regions are shown in yellow and InterPro regions in green. Common (purple) and unique (grey) regions between different splice variants of the gene are also displayed (read more), and at the bottom of the protein view is the protein scale.
LONP1-201
LONP1-202
LONP1-203
LONP1-205
LONP1-208
LONP1-209
LONP1-213
LONP1-215


PROTEIN INFORMATIONi

The protein information section displays alternative protein-coding transcripts (splice variants) encoded by this gene according to the Ensembl database.

The ENSP identifier links to the Ensembl website protein summary, while the ENST identifier links to the Ensembl website transcript summary for the selected splice variant. The data in the UniProt column can be expanded to show links to all matching UniProt identifiers for this protein.

The protein classes assigned to this protein are shown if expanding the data in the protein class column. Parent protein classes are in bold font and subclasses are listed under the parent class.

The Gene Ontology terms assigned to this protein are listed if expanding the Gene ontology column. The length of the protein (amino acid residues according to Ensembl), molecular mass (kDalton), predicted signal peptide (according to a majority of the signal peptide predictors SPOCTOPUS, SignalP 4.0, and Phobius) and the number of predicted transmembrane region(s) (according to MDM) are also reported.
Splice variant UniProt Protein class Gene ontology Length & mass Signal peptide
(predicted)
Transmembrane regions
(predicted)
LONP1-201
ENSP00000353826
ENST00000360614
P36776 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Enzymes
   ENZYME proteins
   Hydrolases
   Peptidases
   Serine-type peptidases
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Disease related genes
Potential drug targets
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Mapped to neXtProt
   neXtProt - Evidence at protein level
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000002 [mitochondrial genome maintenance]
GO:0000166 [nucleotide binding]
GO:0001018 [mitochondrial promoter sequence-specific DNA binding]
GO:0001666 [response to hypoxia]
GO:0003677 [DNA binding]
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:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0005829 [cytosol]
GO:0006508 [proteolysis]
GO:0006515 [protein quality control for misfolded or incompletely synthesized proteins]
GO:0007005 [mitochondrion organization]
GO:0007568 [aging]
GO:0008233 [peptidase activity]
GO:0008236 [serine-type peptidase activity]
GO:0009725 [response to hormone]
GO:0010044 [response to aluminum ion]
GO:0016020 [membrane]
GO:0016787 [hydrolase activity]
GO:0016887 [ATPase activity]
GO:0030163 [protein catabolic process]
GO:0032042 [mitochondrial DNA metabolic process]
GO:0034599 [cellular response to oxidative stress]
GO:0034622 [cellular protein-containing complex assembly]
GO:0042645 [mitochondrial nucleoid]
GO:0042802 [identical protein binding]
GO:0043531 [ADP binding]
GO:0043565 [sequence-specific DNA binding]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0051603 [proteolysis involved in cellular protein catabolic process]
GO:0051880 [G-quadruplex DNA binding]
GO:0070182 [DNA polymerase binding]
GO:0070407 [oxidation-dependent protein catabolic process]
Show all
959 aa
106.5 kDa
No 0
LONP1-202
ENSP00000441523
ENST00000540670
P36776 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Enzymes
   ENZYME proteins
   Hydrolases
   Peptidases
   Serine-type peptidases
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Disease related genes
Potential drug targets
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Mapped to neXtProt
   neXtProt - Evidence at protein level
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000002 [mitochondrial genome maintenance]
GO:0000166 [nucleotide binding]
GO:0001018 [mitochondrial promoter sequence-specific DNA binding]
GO:0001666 [response to hypoxia]
GO:0003677 [DNA binding]
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:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0005829 [cytosol]
GO:0006508 [proteolysis]
GO:0006515 [protein quality control for misfolded or incompletely synthesized proteins]
GO:0007005 [mitochondrion organization]
GO:0008233 [peptidase activity]
GO:0008236 [serine-type peptidase activity]
GO:0016020 [membrane]
GO:0016787 [hydrolase activity]
GO:0016887 [ATPase activity]
GO:0030163 [protein catabolic process]
GO:0032042 [mitochondrial DNA metabolic process]
GO:0034599 [cellular response to oxidative stress]
GO:0042645 [mitochondrial nucleoid]
GO:0042802 [identical protein binding]
GO:0043531 [ADP binding]
GO:0043565 [sequence-specific DNA binding]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0051603 [proteolysis involved in cellular protein catabolic process]
GO:0051880 [G-quadruplex DNA binding]
GO:0070182 [DNA polymerase binding]
GO:0070407 [oxidation-dependent protein catabolic process]
Show all
763 aa
85.6 kDa
No 0
LONP1-203
ENSP00000465585
ENST00000585374
K7EKE6 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000166 [nucleotide binding]
GO:0003677 [DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0005829 [cytosol]
GO:0006508 [proteolysis]
GO:0006515 [protein quality control for misfolded or incompletely synthesized proteins]
GO:0008233 [peptidase activity]
GO:0008236 [serine-type peptidase activity]
GO:0016787 [hydrolase activity]
GO:0016887 [ATPase activity]
GO:0030163 [protein catabolic process]
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]
Show all
845 aa
95.2 kDa
No 0
LONP1-205
ENSP00000468114
ENST00000587365
K7ER56 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0006508 [proteolysis]
GO:0030163 [protein catabolic process]
Show all
210 aa
22.6 kDa
No 0
LONP1-208
ENSP00000468379
ENST00000589473
K7ERR6 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0006508 [proteolysis]
GO:0008233 [peptidase activity]
GO:0008236 [serine-type peptidase activity]
GO:0016787 [hydrolase activity]
GO:0030163 [protein catabolic process]
Show all
264 aa
27.8 kDa
No 0
LONP1-209
ENSP00000468083
ENST00000590206
K7ER27 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0006508 [proteolysis]
GO:0008236 [serine-type peptidase activity]
GO:0016887 [ATPase activity]
GO:0030163 [protein catabolic process]
Show all
242 aa
27.5 kDa
No 0
LONP1-213
ENSP00000465139
ENST00000590729
K7EJE8 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
   MEMSAT-SVM predicted membrane proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000166 [nucleotide binding]
GO:0003677 [DNA binding]
GO:0004176 [ATP-dependent peptidase activity]
GO:0004252 [serine-type endopeptidase activity]
GO:0005524 [ATP binding]
GO:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0005829 [cytosol]
GO:0006508 [proteolysis]
GO:0006515 [protein quality control for misfolded or incompletely synthesized proteins]
GO:0008233 [peptidase activity]
GO:0008236 [serine-type peptidase activity]
GO:0016787 [hydrolase activity]
GO:0016887 [ATPase activity]
GO:0030163 [protein catabolic process]
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]
Show all
829 aa
93.3 kDa
No 0
LONP1-215
ENSP00000468541
ENST00000593119
P36776 [Direct mapping]
Lon protease homolog, mitochondrial
Show all
Enzymes
   ENZYME proteins
   Hydrolases
   Peptidases
   Serine-type peptidases
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Disease related genes
Potential drug targets
Human disease related genes
   Congenital malformations
   Congenital malformations of the musculoskeletal system
Mapped to neXtProt
   neXtProt - Evidence at protein level
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000002 [mitochondrial genome maintenance]
GO:0000166 [nucleotide binding]
GO:0001018 [mitochondrial promoter sequence-specific DNA binding]
GO:0001666 [response to hypoxia]
GO:0003677 [DNA binding]
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:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0005829 [cytosol]
GO:0006508 [proteolysis]
GO:0006515 [protein quality control for misfolded or incompletely synthesized proteins]
GO:0007005 [mitochondrion organization]
GO:0008233 [peptidase activity]
GO:0008236 [serine-type peptidase activity]
GO:0016020 [membrane]
GO:0016787 [hydrolase activity]
GO:0016887 [ATPase activity]
GO:0030163 [protein catabolic process]
GO:0032042 [mitochondrial DNA metabolic process]
GO:0034599 [cellular response to oxidative stress]
GO:0042645 [mitochondrial nucleoid]
GO:0042802 [identical protein binding]
GO:0043531 [ADP binding]
GO:0043565 [sequence-specific DNA binding]
GO:0051131 [chaperone-mediated protein complex assembly]
GO:0051603 [proteolysis involved in cellular protein catabolic process]
GO:0051880 [G-quadruplex DNA binding]
GO:0070182 [DNA polymerase binding]
GO:0070407 [oxidation-dependent protein catabolic process]
Show all
895 aa
100.4 kDa
No 0

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