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Rabbit Anti-C1GALT1C1  antibody (bs-13801R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-13801R
英文名称 C1GALT1C1
中文名称 半乳糖基转移酶2抗体
别    名 HSPC067; 3-galactosyltransferase 2; Beta 1,3 galactosyltransferase 2; Beta1,3 galactosyltransferase 2; C1Gal T2; C1Gal-T2; C1GALT1 specific chaperone 1; C1GALT1-specific chaperone 1; C1galt1c1; C1GalT2; C1GLC_HUMAN; C38H2 L1; C38H2 like protein 1; C38H2-L1; C38H2-like protein 1; C38H2L1; Core 1 beta1; Core 1 beta3 galactosyltransferase specific molecular chaperone; Core 1 beta3-Gal-T2; Core 1 beta3-galactosyltransferase-specific molecular chaperone; Core 1 UDP galactose:N acetylgalactosamine alpha R beta 1,3 galactosyltransferase 2; COSMC; HSPC067; MGC19947; MGC19947; MST143.  
研究领域 肿瘤  细胞生物  信号转导  糖蛋白  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Dog,Pig,Cow,Horse,Sheep)
产品应用 WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 36kDa
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human C1GALT1C1: 181-280/318 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 This gene encodes a type II transmembrane protein that is similar to the core 1 beta1,3-galactosyltransferase 1, which catalyzes the synthesis of the core-1 structure, also known as Thomsen-Friedenreich antigen, on O-linked glycans. This gene product lacks the galactosyltransferase activity itself, but instead acts as a molecular chaperone required for the folding, stability and full activity of the core 1 beta1,3-galactosyltransferase 1. Mutations in this gene have been associated with Tn syndrome. Alternatively spliced transcript variants encoding the same protein have been identified. [provided by RefSeq, Dec 2009]

Function:
Probable chaperone required for the generation of 1 O-glycan Gal-beta1-3GalNAc-alpha1-Ser/Thr (T antigen), which is a precursor for many extended O-glycans in glycoproteins. Probably acts as a specific molecular chaperone assisting the folding/stability of core 1 beta-3-galactosyltransferase (C1GALT1).

Subunit:
Associates with core 1 beta-3-galactosyltransferase (C1GALT1), probably not with the soluble active form.

Subcellular Location:
Membrane; Single-pass type II membrane protein (Potential).

Tissue Specificity:
Ubiquitously expressed. Abundantly expressed in salivary gland, stomach, small intestine, kidney, and testis and at intermediate levels in whole brain, cerebellum, spinal cord, thymus, spleen, trachea, lung, pancreas, ovary, and uterus.

DISEASE:
Defects in C1GALT1C1 are the cause of Tn syndrome (TNSYN) [MIM:300622]. Tn syndrome is a rare autoimmune disease caused by somatic mutation in the C1GALT1C1 gene in which subpopulations of blood cells of all lineages carry an incompletely glycosylated membrane glycoprotein, i.e. the Tn antigen. Since leukocytes and platelets are affected as well as red cells, anemia, leukopenia and thrombocytopenia are features. Tn-polyagglutinability is sometimes associated with leukemia or is a preleukemic state.

Similarity:
Belongs to the glycosyltransferase 31 family. Beta3-Gal-T subfamily.

SWISS:
Q96EU7

Gene ID:
29071

Database links:

Entrez Gene: 29071 Human

Omim: 300611 Human

SwissProt: Q96EU7 Human

Unigene: 643920 Human



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