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Rabbit Anti-DOLK/Gold Conjugated antibody (bs-14406R-Gold)
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說(shuō) 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-14406R-Gold
英文名稱 Rabbit Anti-DOLK/Gold Conjugated antibody
中文名稱 膠體金標(biāo)記的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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 細(xì)胞生物  跨膜蛋白  細(xì)胞膜蛋白  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Chicken, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 59kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DOLK
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 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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