ALG11,天门冬酰胺连接糖基化11抗体

产品编号HZ-7957R
英文名称ALG11
中文名称天门冬酰胺连接糖基化11抗体
别 名Asparagine-linked glycosylation protein 11 homolog; AI849156; alg11; ALG11_HUMAN; Asparagine-linked glycosylation 11; Asparagine-linked glycosylation 11, alpha-1,2-mannosyltransferase homolog (yeast); GT8; UTP14C.
规格价格0.1ml 0.2ml 
说 明 书0.1ml 0.2ml
研究领域细胞生物 免疫学
抗体来源Rabbit
ALG11,天门冬酰胺连接糖基化11抗体克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Dog, Horse,
产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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56kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human ALG11
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.
ALG11,天门冬酰胺连接糖基化11抗体PubMedPubMed
产品介绍background:
Mannosyltransferase involved in the last steps of the synthesis of Man5GlcNAc(2)-PP-dolichol core oligosaccharide on the cytoplasmic face of the endoplasmic reticulum. Catalyzes the addition of the 4th and 5th mannose residues to the dolichol-linked oligosaccharide chain.
Involvement in disease:Defects in ALG11 are the cause of congenital disorder of glycosylation type 1P (CDG1P). 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.

Function:
Mannosyltransferase involved in the last steps of the synthesis of Man5GlcNAc(2)-PP-dolichol core oligosaccharide on the cytoplasmic face of the endoplasmic reticulum. Catalyzes the addition of the 4th and 5th mannose residues to the dolichol-linked oligosaccharide chain.

Subcellular Location:
Endoplasmic reticulum. Endoplasmic reticulum membrane; Multi-pass membrane protein (Probable).

Post-translational modifications:

DISEASE:
Defects in ALG11 are the cause of congenital disorder of glycosylation type 1P (CDG1P) [MIM:613661]. 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.

ALG11,天门冬酰胺连接糖基化11抗体Similarity:
Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 4 subfamily.

Gene ID:
44013

Database links:
Entrez Gene: 440138 Human
Entrez Gene: 207958 Mouse
Entrez Gene: 361174 Rat
Omim: 613666 Human
SwissProt: Q2TAA5 Human
SwissProt: Q3TZM9 Mouse
Unigene: 512963 Human
Unigene: 267439 Mouse
Unigene: 446082 Mouse
Unigene: 37199 Rat

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