Anti-COQ6/FITC Conjugated抗体-抗体-抗体-生物在线
Anti-COQ6/FITC Conjugated抗体

Anti-COQ6/FITC Conjugated抗体

商家询价

产品名称: Anti-COQ6/FITC Conjugated抗体

英文名称: Anti-COQ6/FITC

产品编号: YB--13987R-FITC

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

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 Anti-COQ6/FITC Conjugated抗体

产品编号 YB-13987R-FITC
英文名称 Anti-COQ6/FITC
中文名称 FITC标记的辅酶生物合成单加氧酶COQ6抗体
别    名 CGI-10; Coenzyme Q6 homolog (yeast); Coenzyme Q6 homolog, monooxygenase (S. cerevisiae); Coenzyme Q6 homolog, monooxygenase (yeast); coq6; COQ6_HUMAN; Ubiquinone biosynthesis monooxygenase COQ6.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  细胞凋亡  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Rabbit, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 51kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COQ6
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012]

Subcellular Location:
Golgi apparatus. Cell projection. Note=Localizes to podocyte cell processes.

Tissue Specificity:
Widely epressed.

DISEASE:
Coenzyme Q10 deficiency, primary, 6 (COQ10D6) [MIM:614650]: An autosomal recessive disorder characterized by onset in infancy of severe progressive nephrotic syndrome resulting in end-stage renal failure and sensorineural deafness. Renal biopsy usually shows focal segmental glomerulosclerosis. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the ubiH/COQ6 family.

Database links:

Entrez Gene: 51004 Human

SwissProt: Q9Y2Z9 Human

Unigene: 131555 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications