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Rabbit Anti-DOLK  antibody (bs-14406R)  
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產品編號 bs-14406R
英文名稱 Rabbit Anti-DOLK  antibody
中文名稱 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.  
研究領域 細胞生物  跨膜蛋白  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse (predicted: Human,Rat,Pig,Sheep,Cow,Chicken,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 59kDa
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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