Anti-DPAGT1抗体The protein encoded by this gene is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide

产品编号 yb-14412R
英文名称 Anti-DPAGT1抗体
中文名称 DPAGT1蛋白抗体
别    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.
 Anti-DPAGT1抗体  
说 明 书 0.2ml  
研究领域 细胞生物  免疫学  神经生物学  细胞分化  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 46kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human DPAGT1
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.
 
PubMed PubMed
产品介绍 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.