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    COQ6 Rabbit pAb (bs-25207R)  
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    產品編號 bs-25207R
    英文名稱 COQ6 Rabbit pAb
    中文名稱 輔酶生物合成單加氧酶COQ6抗體
    別    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.  
    研究領域 細胞生物  神經生物學  細胞凋亡  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    克 隆 號
    交叉反應 Mouse,Rat (predicted: Human,Pig,Dog,Horse)
    產品應用 WB=1:500-2000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 51 kDa
    檢測分子量
    細胞定位 細胞漿 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human COQ6 : 401-468/468 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產品介紹 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.

    SWISS:
    Q9Y2Z9

    Gene ID:
    51004

    Database links:

    Entrez Gene: 51004 Human

    SwissProt: Q9Y2Z9 Human

    Unigene: 131555 Human



    產品圖片
    Sample: Lane 1: Mouse Cerebrum tissue lysates Lane 2: Mouse Liver tissue lysates Lane 3: Mouse Heart tissue lysates Lane 4: Rat Cerebrum tissue lysates Lane 5: Rat Liver tissue lysates Lane 6: Rat Heart tissue lysates Primary: Anti-COQ6 (bs-25207R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 51 kDa Observed band size: 51 kDa
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