LONP1

GENERAL INFORMATION

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Gene name

LONP1

Gene description

Lon peptidase 1, mitochondrial

Protein class

Enzymes
Mitochondrial proteins
Predicted intracellular proteins

Predicted localization

Intracellular

Number of transcripts

8
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HUMAN PROTEIN ATLAS INFORMATION

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RNA tissue category

HPA:Expressed in all
GTEx:Expressed in all

Protein evidence

Evidence at protein level

Protein localization

Granular cytoplasmic expression.

ANTIBODY RELIABILITY

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Data reliability
description

Pending RNA-based expert annotation.

Data reliability

Supportive based on 1 antibody.
HPA002192
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RNA AND PROTEIN EXPRESSION SUMMARY

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RNA expression (FPKM) Protein expression (score)
Brain
Endocrine tissues
Bone marrow & immune system
Muscle tissues
Lung
Liver & gallbladder
Pancreas
Gastrointestinal tract
Kidney & urinary bladder
Male tissues
Female tissues
Adipose & soft tissue
Skin
Cerebral cortex

Lymph node

Liver

Duodenum

Colon

Kidney

Testis



PROTEIN EXPRESSION OVERVIEW

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Organ

Expression



RNA EXPRESSION OVERVIEW

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HPA dataset
RNA tissue category:  Expressed in all

Organ

Expression

GTEx dataset
RNA tissue category:  Expressed in all

Organ

Expression



GENE INFORMATION

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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 78.38)

Entrez gene

9361

UniProt

P36776 (UniProt - Evidence at protein level)

neXtProt

NX_P36776

Antibodypedia

LONP1 antibodies


PROTEIN BROWSER

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ENST00000360614
 
ENST00000540670
 
ENST00000585374
 
ENST00000587365
 
ENST00000589473
 
ENST00000590206
 
ENST00000590729
 
ENST00000593119
 
 
 
 


PROTEIN INFORMATION

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Splice variant

Ensembl

UniProt

Protein class

Gene ontology

Length
(aa)

Molecular mass
(kDa)

Signal peptide
(predicted)

Transmembrane regions
(predicted)

LONP1-001 ENSP00000353826
ENST00000360614
P36776
Show all »
Show » Show » 959 106.5 No 0
LONP1-002 ENSP00000441523
ENST00000540670
P36776
Show all »
Show » Show » 763 85.6 No 0
LONP1-003 ENSP00000465585
ENST00000585374
K7EKE6
Show all »
Show » Show » 845 95.2 No 0
LONP1-005 ENSP00000468541
ENST00000593119
P36776
Show all »
Show » Show » 895 100.4 No 0
LONP1-007 ENSP00000465139
ENST00000590729
K7EJE8
Show all »
Show » Show » 829 93.3 No 0
LONP1-014 ENSP00000468083
ENST00000590206
Show » Show » 242 27.5 No 0
LONP1-015 ENSP00000468114
ENST00000587365
K7ER56
Show all »
Show » Show » 210 22.6 No 0
LONP1-016 ENSP00000468379
ENST00000589473
K7ERR6
Show all »
Show » Show » 264 27.8 No 0