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Rabbit Anti-DPAGT1/PE-Cy3 Conjugated antibody (bs-14412R-PE-Cy3)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-14412R-PE-Cy3
英文名稱1 Rabbit Anti-DPAGT1/PE-Cy3 Conjugated antibody
中文名稱 PE-Cy3標(biāo)記的DPAGT1蛋白抗體
別    名 AU021132; Dolichyl phosphate (UDP N acetylglucosamine) acetylglucosaminephosphotransferase 1 (GlcNAc 1 P transferase); DPAGT1; DPAGT2; G1PT; GlcNAc-1-P transferase; Gnpta; GPT; GPT_HUMAN; H2afx; N-acetylglucosamine-1-phosphate transferase; UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  細(xì)胞分化  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)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 46kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DPAGT1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
The protein encoded by this gene is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide pathway for glycoprotein biosynthesis. This enzyme belongs to the glycosyltransferase family 4. This protein is an integral membrane protein of the endoplasmic reticulum. The congenital disorder of glycosylation type Ij is caused by mutation in the gene encoding this enzyme. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the initial step in the synthesis of dolichol-P-P-oligosaccharides.

Subcellular Location:
Endoplasmic reticulum membrane.

DISEASE:
Defects in DPAGT1 are the cause of congenital disorder of glycosylation type 1J (CDG1J) [MIM:608093]. CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of 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.

Similarity:
Belongs to the glycosyltransferase 4 family.

Database links:

Entrez Gene: 1798 Human

Entrez Gene: 13478 Mouse

Omim: 191350 Human

SwissProt: Q9H3H5 Human

SwissProt: P42867 Mouse

Unigene: 524081 Human

Unigene: 18353 Mouse



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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