Lonp1 lon peptidase 1, mitochondrial [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Lonp1provided by MGI
Official Full Name: lon peptidase 1, mitochondrialprovided by MGI
Primary source: MGI:MGI:1921392
See related: Ensembl:ENSMUSG00000041168 AllianceGenome:MGI:1921392
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: LON; Prss15; 1200017E13Rik
Summary: Enables several functions, including ATP hydrolysis activity; ATP-dependent peptidase activity; and DNA binding activity. Acts upstream of or within negative regulation of insulin receptor signaling pathway; proteolysis; and regulation of peptidyl-tyrosine phosphorylation. Located in mitochondrion. Is expressed in several structures, including adrenal medulla; genitourinary system; gut; lung; and submandibular gland primordium. 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: Broad expression in adrenal adult (RPKM 284.2), ovary adult (RPKM 104.2) and 27 other tissues See more
Orthologs: human all
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Genomic context

Location:: 17 29.42 cM; 17 D

Exon count:: 18

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	17	NC_000083.7 (56921297..56933903, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	17	NC_000083.6 (56614297..56626903, complement)

Chromosome 17 - NC_000083.7 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

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Aging of alveolar type 2 cells induced by Lonp1 deficiency exacerbates pulmonary fibrosis.
Title: Aging of alveolar type 2 cells induced by Lonp1 deficiency exacerbates pulmonary fibrosis.
LONP1 alleviates ageing-related renal fibrosis by maintaining mitochondrial homeostasis.
Title: LONP1 alleviates ageing-related renal fibrosis by maintaining mitochondrial homeostasis.
LONP1 ameliorates liver injury and improves gluconeogenesis dysfunction in acute-on-chronic liver failure.
Title: LONP1 ameliorates liver injury and improves gluconeogenesis dysfunction in acute-on-chronic liver failure.
Mitochondrial Lon protease promotes CD4[+] T cell activation by activating the cGAS-STING-TBK1 axis in systemic lupus erythematosus.
Title: Mitochondrial Lon protease promotes CD4(+) T cell activation by activating the cGAS-STING-TBK1 axis in systemic lupus erythematosus.
Decreased LONP1 expression contributes to DNA damage and meiotic defects in oocytes.
Title: Decreased LONP1 expression contributes to DNA damage and meiotic defects in oocytes.
LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease.
Title: LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease.
Sirt3 restricts tumor initiation via promoting LONP1 deacetylation and K63 ubiquitination.
Title: Sirt3 restricts tumor initiation via promoting LONP1 deacetylation and K63 ubiquitination.
LONP1-mediated mitochondrial quality control safeguards metabolic shifts in heart development.
Title: LONP1-mediated mitochondrial quality control safeguards metabolic shifts in heart development.
The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals.
Title: The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals.
Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength.
Title: Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength.

Submit: New GeneRIF Correction See all GeneRIFs (23)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (1)
Targeted (6) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables ADP binding	IEA Inferred from Electronic Annotation more info
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	IDA Inferred from Direct Assay more info	PubMed
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	ISO Inferred from Sequence Orthology more info
enables DNA polymerase binding	IEA Inferred from Electronic Annotation more info
enables DNA polymerase binding	ISO Inferred from Sequence Orthology more info
enables G-quadruplex DNA binding	IEA Inferred from Electronic Annotation more info
enables G-quadruplex DNA binding	ISO Inferred from Sequence Orthology more info
enables PH domain binding	IPI Inferred from Physical Interaction more info	PubMed
enables identical protein binding	IEA Inferred from Electronic Annotation more info
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables insulin receptor substrate binding	IPI Inferred from Physical Interaction more info	PubMed
NOT enables mitochondrial promoter sequence-specific DNA binding	IDA Inferred from Direct Assay more info	PubMed
enables mitochondrial promoter sequence-specific DNA binding	IDA Inferred from Direct Assay more info	PubMed
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	IDA Inferred from Direct Assay more info	PubMed
enables single-stranded RNA binding	IEA Inferred from Electronic Annotation 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	IEA Inferred from Electronic Annotation more info
involved_in mitochondrion organization	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within negative regulation of insulin receptor signaling pathway	IDA Inferred from Direct Assay more info	PubMed
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	ISO Inferred from Sequence Orthology more info
involved_in protein catabolic process	ISS Inferred from Sequence or Structural Similarity 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	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within proteolysis	IDA Inferred from Direct Assay more info	PubMed
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	IDA Inferred from Direct Assay more info	PubMed
involved_in response to aluminum ion	IEA Inferred from Electronic Annotation more info
involved_in response to hormone	IEA Inferred from Electronic Annotation more info
involved_in response to hypoxia	IEA Inferred from Electronic Annotation 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	ISO Inferred from Sequence Orthology more info
located_in mitochondrial nucleoid	IEA Inferred from Electronic Annotation more info
located_in mitochondrial nucleoid	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
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_083058.2

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_028782.2 → NP_083058.2 lon protease homolog, mitochondrial precursor

See identical proteins and their annotated locations for NP_083058.2

Status: VALIDATED

Source sequence(s)

AK160071, BY247676, CK428749

Consensus CDS

CCDS28910.1

UniProtKB/Swiss-Prot

Q3TSK9, Q3TVL2, Q3TXE4, Q3TZW3, Q8CGK3, Q9DBP9

UniProtKB/TrEMBL

Q3V2D0

Related

ENSMUSP00000041814.9, ENSMUST00000047226.10

Conserved Domains (5) summary

smart00464
Location:111 → 188

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

TIGR00763
Location:113 → 936

lon; endopeptidase La

pfam00004
Location:508 → 650

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

cl21455
Location:478 → 529

P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases

cl21678
Location:725 → 938

ChlI; Subunit ChlI of Mg-chelatase