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Anti-ALG11/FITC抗体
产品编号YB7957R-FITC
英文名称Anti-ALG11/FITC
中文名称FITC标记的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.
Anti-ALG11/FITC抗体
说 明 书100ul
研究领域细胞生物 **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.
Anti-ALG11/FITC抗体
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. Anti-ALG11/FITC抗体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.
Similarity:
Anti-ALG11/FITC抗体Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 4 subfamily.
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.