| 英文名称 | Anti-ALG11/FITC |
| 中文名称 | FITC标记的天*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. |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 免疫学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Horse, |
| 产品应用 | IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 56kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 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 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 |
| 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. |
| 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).
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. |
