clk-1 NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: clk-1
Official Full Name: NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase, mitochondrial
Primary source: WormBase:WBGene00000536
Locus tag: CELE_ZC395.2
See related: AllianceGenome:WB:WBGene00000536
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: Enables transcription cis-regulatory region binding activity. Involved in several processes, including determination of adult lifespan; positive regulation of growth rate; and regulation of macromolecule metabolic process. Located in mitochondrion and nucleus. Is expressed widely. Human ortholog(s) of this gene implicated in autosomal recessive distal hereditary motor neuronopathy 9 and primary coenzyme Q10 deficiency 8. Orthologous to human COQ7 (coenzyme Q7, hydroxylase). [provided by Alliance of Genome Resources, Jul 2025]
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Genomic context

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See clk-1 in Genome Data Viewer

Location:: chromosome: III

Exon count:: 5

Sequence:: Chromosome: III; NC_003281.10 (5277894..5279344)

Chromosome III - NC_003281.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_003281.10

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Elevated mitochondrial DNA copy number found in ubiquinone-deficient clk-1 mutants is not rescued by ubiquinone precursor 2-4-dihydroxybenzoate.
Title: Elevated mitochondrial DNA copy number found in ubiquinone-deficient clk-1 mutants is not rescued by ubiquinone precursor 2-4-dihydroxybenzoate.
Loss of ubiquinone biosynthesis is responsible for all phenotypes resulting from loss of CLK-1, including behavioral phenotypes, altered expression of mitochondrial quality control genes, and lifespan.
Title: A single biochemical activity underlies the pleiotropy of the aging-related protein CLK-1.
Nuclear CLK-1 mediates a retrograde signalling pathway that is conserved from Caenorhabditis elegans to humans and is responsive to mitochondrial reactive oxygen species, thus acting as a barometer of oxidative metabolism.
Title: A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
no measurable intrinsic ETC defect exists in clk-1 mitochondria. The data indicate that DMQ(9) specifically inhibits electron transfer from complex I to ubiquinone.
Title: The role of DMQ(9) in the long-lived mutant clk-1.
action of clioquinol on several age-dependent neurodegenerative diseases with distinct etiologies might result from a slowing down of the aging process through action of the drug on CLK-1.
Title: The anti-neurodegeneration drug clioquinol inhibits the aging-associated protein CLK-1.
in maternally resqued animals, the effect of clk-1 mutations on adult lifespan in uncoupled from their effects on development and reproduction
Title: Molecular mechanism of maternal rescue in the clk-1 mutants of Caenorhabditis elegans.
Caenorhabditis elegans clk-1 mutants are sensitive to ubiquinone side-chain length
Title: Sensitivity of Caenorhabditis elegans clk-1 mutants to ubiquinone side-chain length reveals multiple ubiquinone-dependent processes.
Complementation of Escherichia coli ubiF mutation by Caenorhabditis elegans CLK-1.
Title: Complementation of Escherichia coli ubiF mutation by Caenorhabditis elegans CLK-1, a product of the longevity gene of the nematode worm.
Suppressors of the missense mutation of clk-1 were identified; each mutant suppresses a different subset of phenotypes.
Title: Uncoupling the pleiotropic phenotypes of clk-1 with tRNA missense suppressors in Caenorhabditis elegans.
Prolonged life span of Clk mutants cannot be attributed to reduced metabolic rate or an increased activity of the major antioxidant enzymes catalase and SOD.
Title: No reduction of energy metabolism in Clk mutants.

Submit: New GeneRIF Correction See all GeneRIFs (11)

Pathways from PubChem

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General gene information

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Markers

ZC395.2 (e-PCR)
- UniSTS:318716

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables 3-demethoxyubiquinone 3-hydroxylase (NADH) activity	IBA Inferred from Biological aspect of Ancestor more info
enables 3-demethoxyubiquinone 3-hydroxylase (NADH) activity	IEA Inferred from Electronic Annotation more info
enables monooxygenase activity	IEA Inferred from Electronic Annotation more info
enables oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen	IEA Inferred from Electronic Annotation more info
enables transcription cis-regulatory region binding	IDA Inferred from Direct Assay more info	PubMed

Process	Evidence Code	Pubs
involved_in adult behavior	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in cellular respiration	TAS Traceable Author Statement more info	PubMed
involved_in determination of adult lifespan	IBA Inferred from Biological aspect of Ancestor more info
involved_in determination of adult lifespan	IGI Inferred from Genetic Interaction more info	PubMed
involved_in determination of adult lifespan	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of transcription by RNA polymerase II	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in oxidative phosphorylation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of behavior	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of developmental process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of growth rate	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of transcription by RNA polymerase II	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in regulation of reactive oxygen species metabolic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in regulation of reactive oxygen species metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in response to xenobiotic stimulus	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in ubiquinone biosynthetic process	IBA Inferred from Biological aspect of Ancestor more info
involved_in ubiquinone biosynthetic process	IDA Inferred from Direct Assay more info	PubMed
involved_in ubiquinone biosynthetic process	IEA Inferred from Electronic Annotation more info
involved_in ubiquinone biosynthetic process	IMP Inferred from Mutant Phenotype more info	PubMed

Component	Evidence Code	Pubs
located_in extrinsic component of mitochondrial inner membrane	IEA Inferred from Electronic Annotation more info
is_active_in mitochondrial inner membrane	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial inner membrane	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	IEA Inferred from Electronic Annotation more info
is_active_in nucleus	IBA Inferred from Biological aspect of Ancestor more info
located_in nucleus	IDA Inferred from Direct Assay more info	PubMed
located_in nucleus	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase, mitochondrial

NP_498128.1

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_003281.10 Reference assembly

Range

5277894..5279344

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_065727.4 → NP_498128.1 NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase, mitochondrial

See identical proteins and their annotated locations for NP_498128.1

Status: REVIEWED

UniProtKB/Swiss-Prot

P48376

Conserved Domains (1) summary

pfam03232
Location:18 → 187

COQ7; Ubiquinone biosynthesis protein COQ7