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    DOLK Rabbit pAb (bs-14406R)  
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    產(chǎn)品編號 bs-14406R
    英文名稱(chēng) DOLK Rabbit pAb
    中文名稱(chēng) TMEM15/跨膜蛋白15抗體
    別    名 CDG1M; DK; DK1; Dolichol kinase; KIAA1094; DOLK_HUMAN; SEC59; SEC59, YEAST, HOMOLOG OF; TMEM15; Transmembrane protein 15.  
    研究領(lǐng)域 細胞生物  跨膜蛋白  細胞膜蛋白  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Mouse (predicted: Human,Rat,Pig,Sheep,Cow,Chicken,Horse)
    產(chǎn)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 59 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human DOLK: 401-500/538 
    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(chǎn)品介紹 The protein encoded by this gene catalyzes the CTP-mediated phosphorylation of dolichol, and is involved in the synthesis of Dol-P-Man, which is an essential glycosyl carrier lipid for C- and O-mannosylation, N- and O-linked glycosylation of proteins, and for the biosynthesis of glycosyl phosphatidylinositol anchors in endoplasmic reticulum. Mutations in this gene are associated with dolichol kinase deficiency.[provided by RefSeq, Apr 2010]

    Function:
    DOLK belongs to the polyprenol kinase family. Defects in DOLK are the cause of congenital disorder of glycosylation type 1M (CDG1M), also known as dolichol kinase deficiency.

    Subcellular Location:
    Endoplasmic reticulum integral membrane protein

    Tissue Specificity:
    Ubiquitous.

    DISEASE:
    Congenital disorder of glycosylation 1M (CDG1M) [MIM:610768]: A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. CDG1M is a very severe disease with death occurring in early life. Note=The disease is caused by mutations affecting the gene represented in this entry.

    Similarity:
    Belongs to the polyprenol kinase family.

    SWISS:
    Q9UPQ8

    Gene ID:
    22845

    Database links:

    Entrez Gene: 22845 Human

    Omim: 610746 Human

    SwissProt: Q9UPQ8 Human



    產(chǎn)品圖片
    Sample:Muscle (Mouse)Lysate at 40 ug Primary: Anti-DOLK(bs-14406R)at 1/300 dilution Secondary: IRDye800CW Goat Anti-RabbitIgG at 1/20000 dilution Predicted band size: 59kD Observed band size: 59kD
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