Lonp1 lon peptidase 1, mitochondrial [Rattus norvegicus (Norway rat)] - Gene

Summary

Official Symbol: Lonp1provided by RGD
Official Full Name: lon peptidase 1, mitochondrialprovided by RGD
Primary source: RGD:621598
See related: EnsemblRapid:ENSRNOG00000046502 AllianceGenome:RGD:621598
Gene type: protein coding
RefSeq status: PROVISIONAL
Organism: Rattus norvegicus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
Also known as: Lon; Prss15
Summary: Enables ATP-dependent peptidase activity. Involved in several processes, including mitochondrion organization; response to aluminum ion; and response to hypoxia. Located in mitochondrial matrix. Used to study brain ischemia and ischemia. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Biased expression in Adrenal (RPKM 987.4), Kidney (RPKM 361.5) and 9 other tissues See more
Orthologs: human mouse all
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Genomic context

Location:: 9q11

Exon count:: 18

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCr8 (GCF_036323735.1)	9	NC_086027.1 (1534581..1546908, complement)
RS_2023_06	previous assembly	mRatBN7.2 (GCF_015227675.2)	9	NC_051344.1 (1447444..1459771, complement)
106	previous assembly	Rnor_6.0 (GCF_000001895.5)	9	NC_005108.4 (10428853..10441180)

Chromosome 9 - NC_086027.1 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
BioProject: PRJNA238328
Publication: PMID 24510058
Analysis date: Mon Jun 6 17:44:12 2016

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

LONP1 downregulation with ageing contributes to osteoarthritis via mitochondrial dysfunction.
Title: LONP1 downregulation with ageing contributes to osteoarthritis via mitochondrial dysfunction.
LONP1 Regulates Mitochondrial Accumulations of HMGB1 and Caspase-3 in CA1 and PV Neurons Following Status Epilepticus.
Title: LONP1 Regulates Mitochondrial Accumulations of HMGB1 and Caspase-3 in CA1 and PV Neurons Following Status Epilepticus.
upregulation of LonP1 mitigates cardiac injury by preventing oxidative damage of proteins and lipids, preserving mitochondrial redox balance and reprogramming bioenergetics by reducing Complex I content and activity. Mechanisms that promote the upregulation of LonP1 could be beneficial in protecting the myocardium from cardiac stress and limiting I/R injury
Title: Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo.
Lon protease, a major degradation enzyme in mitochondrial matrix, recognized and degraded mitochondrial CBS by specifically targeting at the oxygenated heme group of CBS proteins.
Title: Oxygen-sensitive mitochondrial accumulation of cystathionine β-synthase mediated by Lon protease.
Taken together these findings suggest that Lon protease may be particularly vulnerable to inactivation in conditions associated with GSH depletion and elevated oxidative stress.
Title: Inactivation of brain mitochondrial Lon protease by peroxynitrite precedes electron transport chain dysfunction.
Identification of a newly identified peroxisome-specific isoform of Lon protease, an ATP-dependent protease with chaperone-like activity
Title: Proteomic analysis of rat liver peroxisome: presence of peroxisome-specific isozyme of Lon protease.
changes in nuclear gene expression (Lon) triggered by ER stress had the potential to impact important mitochondrial processes such as assembly and/or degradation of cytochrome c oxidase
Title: Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease.

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

RH143400 (e-PCR)
- UniSTS:232647

RH141449 (e-PCR)
- UniSTS:223443

Gene Ontology Provided by RGD

Function	Evidence Code	Pubs
enables ADP binding	ISO Inferred from Sequence Orthology more info
enables ATP binding	IEA Inferred from Electronic Annotation more info
enables ATP binding	ISO Inferred from Sequence Orthology more info
enables ATP hydrolysis activity	IEA Inferred from Electronic Annotation more info
enables ATP hydrolysis activity	ISO Inferred from Sequence Orthology more info
enables ATP-dependent peptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables ATP-dependent peptidase activity	IDA Inferred from Direct Assay more info	PubMed
enables ATP-dependent peptidase activity	IEA Inferred from Electronic Annotation more info
enables ATP-dependent peptidase activity	ISO Inferred from Sequence Orthology more info
enables DNA polymerase binding	ISO Inferred from Sequence Orthology more info
enables G-quadruplex DNA binding	ISO Inferred from Sequence Orthology more info
enables PH domain binding	ISO Inferred from Sequence Orthology more info
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables insulin receptor substrate binding	ISO Inferred from Sequence Orthology more info
NOT enables mitochondrial promoter sequence-specific DNA binding	ISO Inferred from Sequence Orthology more info
enables mitochondrial promoter sequence-specific DNA binding	ISO Inferred from Sequence Orthology more info
enables sequence-specific DNA binding	IEA Inferred from Electronic Annotation more info
enables sequence-specific DNA binding	ISO Inferred from Sequence Orthology more info
enables serine-type endopeptidase activity	IEA Inferred from Electronic Annotation more info
enables single-stranded DNA binding	IBA Inferred from Biological aspect of Ancestor more info
enables single-stranded DNA binding	ISO Inferred from Sequence Orthology more info
enables single-stranded RNA binding	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in cellular response to oxidative stress	IEA Inferred from Electronic Annotation more info
involved_in cellular response to oxidative stress	ISO Inferred from Sequence Orthology more info
involved_in chaperone-mediated protein complex assembly	IBA Inferred from Biological aspect of Ancestor more info
involved_in chaperone-mediated protein complex assembly	IEA Inferred from Electronic Annotation more info
involved_in mitochondrion organization	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrion organization	IEP Inferred from Expression Pattern more info	PubMed
involved_in mitochondrion organization	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within negative regulation of insulin receptor signaling pathway	ISO Inferred from Sequence Orthology more info
involved_in oxidation-dependent protein catabolic process	IEA Inferred from Electronic Annotation more info
involved_in oxidation-dependent protein catabolic process	ISO Inferred from Sequence Orthology more info
involved_in protein catabolic process	IDA Inferred from Direct Assay more info	PubMed
involved_in protein catabolic process	ISO Inferred from Sequence Orthology more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	IEA Inferred from Electronic Annotation more info
involved_in protein-containing complex assembly	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within proteolysis	ISO Inferred from Sequence Orthology more info
involved_in proteolysis involved in protein catabolic process	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within regulation of peptidyl-tyrosine phosphorylation	ISO Inferred from Sequence Orthology more info
involved_in response to aluminum ion	IEP Inferred from Expression Pattern more info	PubMed
involved_in response to hormone	IEP Inferred from Expression Pattern more info	PubMed
involved_in response to hypoxia	IEP Inferred from Expression Pattern more info	PubMed
involved_in response to hypoxia	ISO Inferred from Sequence Orthology more info

Component	Evidence Code	Pubs
located_in cytosol	IEA Inferred from Electronic Annotation more info
located_in cytosol	ISO Inferred from Sequence Orthology more info
is_active_in mitochondrial matrix	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial matrix	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
located_in mitochondrial matrix	ISO Inferred from Sequence Orthology more info
located_in mitochondrial nucleoid	ISO Inferred from Sequence Orthology more info
is_active_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in nucleoplasm	IEA Inferred from Electronic Annotation more info
located_in nucleoplasm	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: lon protease homolog, mitochondrial

Names: LONP; lon protease-like protein; mitochondrial ATP-dependent protease Lon; protease, serine, 15; serine protease 15

NP_596895.1

EC 3.4.21.53

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_133404.1 → NP_596895.1 lon protease homolog, mitochondrial precursor

See identical proteins and their annotated locations for NP_596895.1

Status: PROVISIONAL

Source sequence(s)

AB064323

UniProtKB/Swiss-Prot

Q924S5

UniProtKB/TrEMBL

A0A8I6AS66, A6KQW3

Related

ENSRNOP00000066618.2, ENSRNOT00000074253.3

Conserved Domains (5) summary

smart00464
Location:111 → 188

LON; Found in ATP-dependent protease La (LON)

TIGR00763
Location:113 → 937

lon; endopeptidase La

pfam00004
Location:509 → 651

AAA; ATPase family associated with various cellular activities (AAA)

cl21455
Location:479 → 530

P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases

cl21678
Location:727 → 938

ChlI; Subunit ChlI of Mg-chelatase