COQ3 - coenzyme Q3, methyltransferase Gene

中文名称：辅酶 Q3，甲基转移酶

种属: Homo sapiens

同用名: DHHBMT; bA9819.1; DHHBMTASE; UG0215E05

基因 ID: 51805 | 基因类型: protein coding

关于 COQ3

Cytogenetic location: 6q16.2 Genomic coordinates (GRCh38): 6:99,369,401-99,394,195 (from NCBI)

This gene has 3 transcripts (splice variants) and 203 orthologues. Ubiquitous expression in heart (RPKM 8.1), brain (RPKM 3.9) and 25 other tissues.

功能概要

泛醌，也称为辅酶 Q 或 Q，是真核生物和原核生物电子传输途径的重要组成部分 (Jonassen 和 Clarke，2000 [PubMed 10777520]) 。这种脂质由疏水性类异戊二烯尾部和醌头基组成。尾巴的长度因生物体而异，但其目的是将辅酶 Q 固定在细胞膜上。醌头基负责呼吸链中辅酶 Q 的活性。酿酒酵母 COQ3 基因编码辅酶 Q 生物合成途径中 2 个步骤所需的 O-甲基转移酶。该酶甲基化早期辅酶 Q 中间体 3,4-二羟基-5-聚异戊二烯苯甲酸，以及最终中间体途径，将去甲基泛醌转化为辅酶 Q。COQ3 基因产物还能够甲基化独特的原核早期中间体 2-羟基-6-聚异戊二烯苯酚。[OMIM 提供，2008 年 3 月]

Ubiquinone, also known as coenzyme Q, or Q, is a critical component of the electron transport pathways of both eukaryotes and prokaryotes (Jonassen and Clarke, 2000 [PubMed 10777520]). This lipid consists of a hydrophobic isoprenoid tail and a quinone head group. The tail varies in length depending on the organism, but its purpose is to anchor coenzyme Q to the membrane. The quinone head group is responsible for the activity of coenzyme Q in the respiratory chain. The S. cerevisiae COQ3 gene encodes an O-methyltransferase required for 2 steps in the biosynthetic pathway of coenzyme Q. This Enzyme methylates an early coenzyme Q intermediate, 3,4-dihydroxy-5-polyprenylbenzoic acid, as well as the final intermediate in the pathway, converting demethyl-ubiquinone to coenzyme Q. The COQ3 gene product is also capable of methylating the distinct prokaryotic early intermediate 2-hydroxy-6-polyprenyl phenol.[supplied by OMIM, Mar 2008]

COQ3 基因产物(1)

mRNA	Protein	Name
NM_017421.4	NP_059117.3	ubiquinone biosynthesis O-methyltransferase, mitochondrial

基因本体论

分子功能
生物过程
细胞组分

分子功能 GO 注释	逻辑证据	参考文献	来源
enables 3-demethylubiquinol-n 3-O-methyltransferase activity	IDA IDA: 通过直接分析推断	10777520	GOA
enables O-methyltransferase activity	IGI IGI: 通过遗传相互作用推断	10777520	GOA
enables protein binding	IPI IPI: 通过物理相互作用推断	27499296	GOA

生物过程 GO 注释	逻辑证据	参考文献	来源
involved in glycerol metabolic process	IGI IGI: 通过遗传相互作用推断	10777520	GOA
involved in ubiquinone biosynthetic process	IGI IGI: 通过遗传相互作用推断	10777520	GOA

细胞组分 GO 注释	逻辑证据	参考文献	来源
located in mitochondrial inner membrane	IDA IDA: 通过直接分析推断	27499296	GOA
located in mitochondrion	IDA IDA: 通过直接分析推断	10777520	GOA
part of ubiquinone biosynthesis complex	IPI IPI: 通过物理相互作用推断	27499296	GOA

EXP：通过实验结果推断 IDA：通过直接分析推断 IPI：通过物理相互作用推断 IMP：通过突变表型推断 IGI：通过遗传相互作用推断 IEP：通过表达模式推断

COQ3 蛋白结构

Methyltransf_23

0
100
200
300
369 a.a.

蛋白主名

其他名称

ubiquinone biosynthesis O-methyltransferase, mitochondrial

2-polyprenyl-6-hydroxyphenol methylase

COQ3 蛋白互作信息

分类	蛋白名称	蛋白编号	互作蛋白	互作蛋白种属	互作蛋白编号	实验方法	参考文献
种属内	COQ3	Q9NZJ6	COQ7	Homo sapiens	Q99807	Anti Tag CoIP	27499296
种属内	COQ3	Q9NZJ6	COQ7	Homo sapiens	Q99807	Pull Down	27499296
种属内	COQ3	Q9NZJ6	NDRG4	Homo sapiens	Q9ULP0-2	Validated Y2H	32296183
种属内	COQ3	Q9NZJ6	COQ4	Homo sapiens	Q9Y3A0	Pull Down	27499296
种属内	COQ3	Q9NZJ6	COQ5	Homo sapiens	Q5HYK3	Pull Down	27499296
种属内	COQ3	Q9NZJ6	COQ5	Homo sapiens	Q5HYK3	Anti Tag CoIP	27499296
种属内	COQ3	Q9NZJ6	NFS1	Homo sapiens	Q9Y697	Anti Tag CoIP	27499296
种属内	COQ3	Q9NZJ6	COQ6	Homo sapiens	Q9Y2Z9	Pull Down	27499296
种属内	COQ3	Q9NZJ6	COQ6	Homo sapiens	Q9Y2Z9	Anti Tag CoIP	27499296

种属间: 跨种属相互作用 种属内: 同种属相互作用

关联疾病

疾病名称

别名

Coenzyme Q10 Deficiency, Primary, 6

Familial Steroid-Resistant Nephrotic Syndrome With Sensorineural Deafness	COQ10D6
Primary Coenzyme Q10 Deficiency 6	Familial Steroid-Resistant Nephrotic Syndrome With Sensorineural Hearing Loss
Srns With Sensorineural Deafness	Steroid-Resistant Nephrotic Syndrome With Sensorineural Deafness
Coenzyme Q10 Deficiency, Primary, Type 6

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

Cleft Soft Palate

Cleft Velum	Cleft Velum Palatinum
Soft Cleft Palate	Soft Palate Perforation

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

疾病名称	别名
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

Cat. No.	产品名	作用方式	纯度	是否罕见病
HY-RS03039	COQ3 Human Pre-designed siRNA Set A	Pre-designed Sets	/	否

直系同源

种属	基因名	来源	基因 ID
Rattus norvegicus	COQ3	RGD	RGD:2380
Canis familiaris	COQ3	VGNC	VGNC:39518
Felis catus	COQ3	VGNC	VGNC:61095
Bos taurus	COQ3	VGNC	VGNC:27612
Mus musculus	COQ3	MGD	MGI:101813
Macaca mulatta	COQ3	VGNC	VGNC:104371

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