lonp-1 Lon protease homolog, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: lonp-1
Official Full Name: Lon protease homolog, mitochondrial
Primary source: WormBase:WBGene00016391
Locus tag: CELE_C34B2.6
See related: AllianceGenome:WB:WBGene00016391
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Caenorhabditis elegans (strain: Bristol N2)
Lineage: Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
Summary: Predicted to enable ATP-dependent peptidase activity and single-stranded DNA binding activity. Involved in protein catabolic process. Predicted to be located in mitochondrion. Predicted to be active in mitochondrial matrix. Is expressed in several structures, including head; hypodermis; intestine; pharynx; and reproductive system. 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]
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Genomic context

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See lonp-1 in Genome Data Viewer

Location:: chromosome: I

Exon count:: 13

Sequence:: Chromosome: I; NC_003279.8 (10676922..10682374, complement)

Chromosome I - NC_003279.8 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_003279.8

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Mitochondrial p38 Mitogen-Activated Protein Kinase: Insights into Its Regulation of and Role in LONP1-Deficient Nematodes.
Title: Mitochondrial p38 Mitogen-Activated Protein Kinase: Insights into Its Regulation of and Role in LONP1-Deficient Nematodes.
LONP-1 and ATFS-1 sustain deleterious heteroplasmy by promoting mtDNA replication in dysfunctional mitochondria.
Title: LONP-1 and ATFS-1 sustain deleterious heteroplasmy by promoting mtDNA replication in dysfunctional mitochondria.

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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

C34B2.10 (e-PCR)
- UniSTS:320692

C34B2.6 (e-PCR)
- UniSTS:309235

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables ATP binding	IEA Inferred from Electronic Annotation more info
enables ATP hydrolysis activity	IEA Inferred from Electronic Annotation more info
enables ATP-dependent peptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables ATP-dependent peptidase activity	IEA Inferred from Electronic Annotation more info
enables DNA binding	IEA Inferred from Electronic Annotation more info
enables sequence-specific DNA binding	IEA Inferred from Electronic Annotation more info
enables serine-type endopeptidase activity	IEA Inferred from Electronic Annotation more info
enables serine-type peptidase activity	IEA Inferred from Electronic Annotation more info
enables single-stranded DNA binding	IBA Inferred from Biological aspect of Ancestor more info

Process	Evidence Code	Pubs
involved_in IRE1-mediated unfolded protein response	HEP more info	PubMed
involved_in cellular response to oxidative stress	IEA Inferred from Electronic Annotation 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 oxidation-dependent protein catabolic process	IEA Inferred from Electronic Annotation more info
involved_in protein catabolic process	IEA Inferred from Electronic Annotation more info
involved_in protein catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
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 proteolysis	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
is_active_in mitochondrial matrix	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: Lon protease homolog, mitochondrial

NP_492796.1

Confirmed by transcript evidence

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

NC_003279.8 Reference assembly

Range

10676922..10682374 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_060395.8 → NP_492796.1 Lon protease homolog, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_492796.1

Status: REVIEWED

UniProtKB/Swiss-Prot

O44952

Conserved Domains (5) summary

smart00464
Location:89 → 151

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

TIGR00763
Location:90 → 970

lon; endopeptidase La

pfam00004
Location:508 → 646

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

cl21455
Location:465 → 531

P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases

cl21678
Location:761 → 970

ChlI; Subunit ChlI of Mg-chelatase