COQ7 - coenzyme Q7, hydroxylase Gene

Homo sapiens

Also known as CAT5; CLK1; CLK-1; COQ10D8

Gene ID: 10229 | Gene type: protein coding

About COQ7

Cytogenetic location: 16p12.3 Genomic coordinates (GRCh38): 16:19,067,614-19,083,097 (from NCBI)

This gene has 11 transcripts (splice variants), 217 orthologues and is associated with 1 phenotype. Ubiquitous expression in prostate (RPKM 5.8), thyroid (RPKM 5.3) and 25 other tissues.

Summary

The protein encoded by this gene is similar to a mitochondrial di-iron containing hydroxylase in Saccharomyces cerevisiae that is involved with ubiquinone biosynthesis. Mutations in the yeast gene lead to slower development and longer life span. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2010]

COQ7 Products(9)

mRNA	Protein	Name
NM_001190983.2	NP_001177912.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 2
NM_001370489.1	NP_001357418.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 3
NM_001370490.1	NP_001357419.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 4
NM_001370491.1	NP_001357420.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 5
NM_001370492.1	NP_001357421.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 2
NM_001370493.1	NP_001357422.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 2
NM_001370494.1	NP_001357423.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 2
NM_001370495.1	NP_001357424.1	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 6
NM_016138.5	NP_057222.2	5-demethoxyubiquinone hydroxylase, mitochondrial isoform 1

COQ7 Protein Structure

COQ7

0
100
200
217 a.a.

Protein Preferred Names

Protein Names

5-demethoxyubiquinone hydroxylase, mitochondrial

COQ7 coenzyme Q, 7 homolog ubiquinone

Recombinant COQ7 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70096	COQ7 Protein, Human (HEK293, His)	Q99807 (S37-L217)	≥95%

Related Diseases

Diseases

Alias

Coenzyme Q10 Deficiency, Primary, 8

COQ10D8

Primary Coenzyme Q10 Deficiency 8

Coenzyme Q10 Deficiency, Primary, 7

Neonatal Encephalomyopathy-Cardiomyopathy-Respiratory Distress Syndrome	COQ10D7
Primary Coenzyme Q10 Deficiency 7	Coq4-Related Neonatal Encephalomyopathy
Coenzyme Q10 Deficiency, Primary, Type 7

Coenzyme Q10 Deficiency Disease

Coenzyme Q10 Deficiency	Coq10 Deficiency
Primary Coenzyme Q10 Deficiency	Coenzyme Q Deficiency
Coq Deficiency	Primary Coq10 Deficiency
Ubiquinone Deficiency	Coenzyme Q10 Deficiency, Primary
Coq10 Deficiency, Primary

Coenzyme Q10 Deficiency, Primary, 4

Scar9	Spinocerebellar Ataxia, Autosomal Recessive 9
Autosomal Recessive Ataxia Due To Ubiquinone Deficiency	COQ10D4
Arca2	Autosomal Recessive Cerebellar Ataxia Type 2
Primary Coenzyme Q10 Deficiency 4	Autosomal Recessive Ataxia Due To Coenzyme Q10 Deficiency
Autosomal Recessive Spinocerebellar Ataxia Type 9	Autosomal Recessive Spinocerebellar Ataxia 9
Spinocerebellar Ataxia Autosomal Recessive 9	Coenzyme Q10 Deficiency, Primary, Type 4
Ataxia, Spinocerebellar, Autosomal Recessive, Type 9

Mitochondrial Myopathy

Mitochondrial Myopathies	Mitochondrial Cytopathy
Myopathies In Mitochondrial Disorders

Mitochondrial Complex I Deficiency, Nuclear Type 1

Mitochondrial Complex I Deficiency	Nadh:Q(1) Oxidoreductase Deficiency
MC1DN1	Nadh-Coenzyme Q Reductase Deficiency
Isolated Mitochondrial Respiratory Chain Complex I Deficiency	Isolated Nadh-Coenzyme Q Reductase Deficiency
Isolated Nadh-Coq Reductase Deficiency	Isolated Nadh-Ubiquinone Reductase Deficiency
Mitochondrial Nadh Dehydrogenase Component Of Complex I, Deficiency Of	Nuclear Type Mitochondrial Complex I Deficiency 1
Isolated Complex I Deficiency	Complex 1 Mitochondrial Respiratory Chain Deficiency
Nadh Coenzyme Q Reductase Deficiency	Complex I Mitochondrial Respiratory Chain Deficiency
Deficiency Of Mitochondrial Nadh Dehydrogenase Component Of Complex I	Nadh:Ubiquinone Oxidoreductase Deficiency
Complex I, Mitochondrial Respiratory Chain, Deficiency Of

Leigh Syndrome

Leigh Disease	Infantile Subacute Necrotizing Encephalopathy
Leigh Syndrome Due To Mitochondrial Complex Iv Deficiency	LS
Sne	Leigh'S Disease
Leigh Syndrome Due To Mitochondrial Complex I Deficiency	Necrotizing Encephalopathy, Infantile Subacute, Of Leigh
Subacute Necrotizing Encephalomyelopathy	Necrotizing Encephalopathy Infantile Subacute Of Leigh
Leigh Syndrome Due To Mitochondrial Complex Iii Deficiency	Infantile Necrotizing Encephalomyelopathy
Juvenile Subacute Necrotizing Encephalomyelopathy	Leigh'S Necrotizing Encephalopathy
Subacute Necrotizing Encephalopathy	Juvenile Subacute Necrotizing Encephalopathy
Leigh Syndrome Due To Mitochondrial Complex Ii Deficiency	Leigh Syndrome Due To Mitochondrial Complex V Deficiency
Encephalopathy, Subacute Necrotizing, Infantile	Encephalopathy, Subacute Necrotizing, Juvenile
Maternally Inherited Leigh Syndrome	Subacute Necrotising Encephalomyelopathy
Subacute Necrotising Encephalopathy

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Bioactive Molecules (3)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-133033	COQ7-IN-1	Inhibitor	99.98%	Unkown
HY-138429	COQ7-IN-2	Inhibitor	99.48%	Unkown
HY-RS03043	COQ7 Human Pre-designed siRNA Set A		/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Felis catus	COQ7	VGNC	VGNC:61098
Bos taurus	COQ7	VGNC	VGNC:27615
Mus musculus	COQ7	MGD	MGI:107207
Rattus norvegicus	COQ7	RGD	RGD:2381
Canis familiaris	COQ7	VGNC	VGNC:39522
Others	COQ7	NCBI

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