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Rabbit Anti-DOLK/PE-Cy7 Conjugated antibody (bs-14406R-PE-Cy7)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-14406R-PE-Cy7
英文名称1 Rabbit Anti-DOLK/PE-Cy7 Conjugated antibody
中文名称 PE-Cy7标记的TMEM15/跨膜蛋白15抗体
别    名 CDG1M; DK; DK1; Dolichol kinase; KIAA1094; DOLK_HUMAN; SEC59; SEC59, YEAST, HOMOLOG OF; TMEM15; Transmembrane protein 15.  
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  跨膜蛋白  细胞膜蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,  (predicted: Human, Rat, Chicken, Pig, Cow, Horse, Sheep, )
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 59kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DOLK
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 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.

Database links:

Entrez Gene: 22845 Human

Omim: 610746 Human

SwissProt: Q9UPQ8 Human



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