COQ6 Antibody

中文名称：

COQ6兔多克隆抗体
货号：

CSB-PA897098LA01HU
规格：

￥440
图片：
Western Blot
Positive WB detected in: Mouse heart tissue, Mouse liver tissue
All lanes: COQ6 antibody at 3μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
predicted band size: 51, 49, 43 kDa
observed band size: 51, 49 kDa

Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA897098LA01HU at dilution of 1:100
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产品名称：

Rabbit anti-Homo sapiens (Human) COQ6 Polyclonal antibody
Uniprot No.：

Q9Y2Z9
基因名：

COQ6
别名：

CGI-10 antibody; Coenzyme Q6 homolog (yeast) antibody; Coenzyme Q6 homolog; monooxygenase (S. cerevisiae) antibody; Coenzyme Q6 homolog; monooxygenase (yeast) antibody; coq6 antibody; COQ6_HUMAN antibody; Ubiquinone biosynthesis monooxygenase COQ6 antibody
宿主：

Rabbit
反应种属：

Human, Mouse
免疫原：

Recombinant Human Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial protein (208-339AA)
免疫原种属：

Homo sapiens (Human)

标记方式：

Non-conjugated

本页面中的产品，COQ6 Antibody (CSB-PA897098LA01HU)，的标记方式是Non-conjugated。对于COQ6 Antibody，我们还提供其他标记。见下表：

标记方式	货号	产品名称	应用
HRP	CSB-PA897098LB01HU	COQ6 Antibody, HRP conjugated	ELISA
FITC	CSB-PA897098LC01HU	COQ6 Antibody, FITC conjugated
Biotin	CSB-PA897098LD01HU	COQ6 Antibody, Biotin conjugated	ELISA

克隆类型：

Polyclonal
抗体亚型：

IgG
纯化方式：

>95%, Protein G purified
浓度：

It differs from different batches. Please contact us to confirm it.
保存缓冲液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
产品提供形式：

Liquid
应用范围：

ELISA, WB, IHC
推荐稀释比：

Application Recommended Dilution

WB 1:500-1:5000

IHC 1:20-1:200
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
储存条件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
货期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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功能：

FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-hexaprenyl-4-hydroxybenzoic acid (HHB) to 3-hexaprenyl-4,5-dihydroxybenzoic acid (DHHB). The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. Is able to perform the deamination reaction at C4 of 3-hexaprenyl-4-amino-5-hydroxybenzoic acid (HHAB) to produce DHHB when expressed in yeast cells lacking COQ9, even if utilization of para-aminobenzoic acid (pABA) involving C4-deamination seems not to occur in bacteria, plants and mammals, where only C5 hydroxylation of HHB has been shown.
基因功能参考文献：
1. Primary CoQ10 deficiency due to COQ6 mutations should be considered in children presenting with both Steroid-Resistant Focal Segmental Glomerulosclerosis and sensorineural hearing loss. An early diagnosis of COQ6 mutations is essential because the condition is treatable when CoQ10 supplementation is started at the early stage. PMID: 28117207
2. Data indicate a heterozygous loss-of-function coenzyme Q10 (CoQ10) biosynthesis monooxygenase 6 gene (COQ6)missense mutation in familial schwannomatosis. PMID: 24763291
3. COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness PMID: 21540551
相关疾病：

Coenzyme Q10 deficiency, primary, 6 (COQ10D6)
亚细胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. Golgi apparatus. Cell projection.
蛋白家族：

UbiH/COQ6 family
组织特异性：

Widely expressed.
数据库链接：

HGNC: 20233

OMIM: 614647

KEGG: hsa:51004

STRING: 9606.ENSP00000333946

UniGene: Hs.131555

COQ6 Antibody

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Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:20-1:200