COQ5 - coenzyme Q5, methyltransferase Gene

Homo sapiens

Also known as COQ10D9

Gene ID: 84274 | Gene type: protein coding

About COQ5

Cytogenetic location: 12q24.31 Genomic coordinates (GRCh38): 12:120,503,279-120,529,158 (from NCBI)

This gene has 8 transcripts (splice variants), 203 orthologues, 4 paralogues and is associated with 2 phenotypes. Ubiquitous expression in kidney (RPKM 15.5), liver (RPKM 13.7) and 25 other tissues.

Summary

Enables 2-octaprenyl-6-methoxy-1,4-benzoquinone methylase activity. Involved in methylation and ubiquinone biosynthetic process. Located in mitochondrial matrix. Part of protein-containing complex. Colocalizes with mitochondrial inner membrane. Implicated in primary coenzyme Q10 deficiency 9. [provided by Alliance of Genome Resources, Apr 2022]

COQ5 Products(1)

mRNA	Protein	Name
NM_032314.4	NP_115690.3	2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables 2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity	IDA IDA: Inferred from direct assay	25152161	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	25152161	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in methylation	IDA IDA: Inferred from direct assay	25152161	GOA
involved in ubiquinone biosynthetic process	IGI IGI: Inferred from genetic interaction	25152161	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
colocalizes with mitochondrial inner membrane	IDA IDA: Inferred from direct assay	25152161	GOA
located in mitochondrial inner membrane	IDA IDA: Inferred from direct assay	27499296	GOA
located in mitochondrial matrix	IDA IDA: Inferred from direct assay	25152161	GOA
part of protein-containing complex	IDA IDA: Inferred from direct assay	25152161	GOA
part of ubiquinone biosynthesis complex	IPI IPI: Inferred from physical interaction	27499296	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

COQ5 Protein Structure

Ubie_methyltran

0
100
200
300
327 a.a.

Protein Preferred Names

Protein Names

2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial

coenzyme Q5 homolog, methyltransferase

COQ5 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	COQ5	Q5HYK3	COQ3	Homo sapiens	Q9NZJ6	Pull Down	27499296
Intra	COQ5	Q5HYK3	COQ3	Homo sapiens	Q9NZJ6	Anti Tag CoIP	27499296
Intra	COQ5	Q5HYK3	COQ7	Homo sapiens	Q99807	Anti Tag CoIP	27499296
Intra	COQ5	Q5HYK3	COQ7	Homo sapiens	Q99807	Pull Down	27499296
Intra	COQ5	Q5HYK3	COQ4	Homo sapiens	Q9Y3A0	Pull Down	27499296
Intra	COQ5	Q5HYK3	COQ6	Homo sapiens	Q9Y2Z9	Anti Tag CoIP	27499296
Intra	COQ5	Q5HYK3	COQ6	Homo sapiens	Q9Y2Z9	Pull Down	27499296
Intra	COQ5	Q5HYK3	COQ9	Homo sapiens	O75208	Pull Down	27499296
Intra	COQ5	Q5HYK3	COQ9	Homo sapiens	O75208	Anti Tag CoIP	27499296

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases

Alias

Coenzyme Q10 Deficiency, Primary, 9

COQ10D9

Primary Coenzyme Q10 Deficiency 9

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

Immunodeficiency 24

Severe Combined Immunodeficiency Due To Ctps1 Deficiency	IMD24
Scid Due To Ctps1 Deficiency	Immunodeficiency, Type 24

Cardiomyopathy, Dilated, 1hh

Dilated Cardiomyopathy 1hh	CMD1HH
Cardiomyopathy, Dilated 1hh	Cardiomyopathy, Dilated, Type 1hh

Nephrotic Syndrome, Type 9

NPHS9	Nephrotic Syndrome Type 9
Nephrotic Syndrome 9

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

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

Spondyloepiphyseal Dysplasia, Nishimura Type

SEDN	Spondyloepiphyseal Dysplasia Nishimura Type

Multiple Acyl-Coa Dehydrogenase Deficiency

MADD	Ethylmalonic-Adipicaciduria
Ema	Glutaric Acidemia Iia
Glutaric Acidemia Iib	Ga Ii
Glutaric Acidemia Iic	Glutaric Acidemia Type 2
Glutaric Acidemia Ii	Glutaric Aciduria Ii
Electron Transfer Flavoprotein Deficiency	Glutaric Aciduria Type 2
Mad Deficiency	Glutaric Acidemia Type Ii
Glutaric Aciduria 2	Etfa Deficiency
Etfb Deficiency	Etfdh Deficiency
Multiple Acyl Coenzyme A Dehydrogenase Deficiency	Ga2
Electron Transfer Flavoprotein Ubiquinone Oxidoreductase Deficiency	Electron Transfer Flavoprotein Dehydrogenase Deficiency
Ga 2	Glutaric Acidemia 2
Glutaric Acidemia, Type 2	Glutaric Aciduria, Type 2
Mad	Multiple Fad Dehydrogenase Deficiency
Ethylmalonic Adipic Aciduria	Glutaricaciduria Ii
Glutaric Aciduria 2a	GA2A
Gaiia	Glutaricaciduria Iia
Glutaric Aciduria 2b	GA2B
Gaiib	Glutaricaciduria Iib
Glutaric Aciduria 2c	GA2C
Gaiic	Glutaricaciduria Iic
Glutaricaciduria, Type Iia	Glutaric Acidemia Type 2a
Glutaric Acidemia Type 2c	Glutaric Aciduria Iia
Glutaric Aciduria Iib	Glutaric Aciduria Iic

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

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 (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS03041	COQ5 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Bos taurus	COQ5	VGNC	VGNC:27613
Felis catus	COQ5	VGNC	VGNC:80434
Mus musculus	COQ5	MGD	MGI:1098643
Rattus norvegicus	COQ5	RGD	RGD:1310857
Canis familiaris	COQ5	VGNC	VGNC:39520
Macaca mulatta	COQ5	VGNC	VGNC:81376

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