天门冬酰胺连接糖基化11抗体
规格：1尘驳/1尘濒
英文名： ALG11
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);
分子量： 56kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ALG11
交叉反应：Human, Mouse, Rat, Dog, Horse,
细胞定位：细胞浆 细胞膜
产天门冬酰胺连接糖基化11抗体品介绍：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天门冬酰胺连接糖基化11抗体 reticulum. Endoplasmic reticulum membrane; Multi-pass membrane protein (Probable). 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天门冬酰胺连接糖基化11抗体 of cell functions. 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.
天门冬酰胺连接糖基化11抗体产物应用：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.
研究领域：细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid