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Rabbit Anti-DPAGT1/Gold Conjugated antibody (bs-14412R-Gold)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-14412R-Gold
英文名稱 Rabbit Anti-DPAGT1/Gold Conjugated antibody
中文名稱 膠體金標記的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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 細胞生物  免疫學  神經生物學  細胞分化  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 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 46kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/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 0.02M 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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