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Rabbit Anti-C1GALT1C1/RBITC Conjugated antibody (bs-13801R-RBITC)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-13801R-RBITC
英文名稱(chēng)1 Rabbit Anti-C1GALT1C1/RBITC Conjugated antibody
中文名稱(chēng) 羅丹明(RBITC)標(biāo)記的半乳糖基轉(zhuǎn)移酶2抗體
別    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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  糖蛋白  新陳代謝  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 36kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human C1GALT1C1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
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.

Database links:

Entrez Gene: 29071 Human

Omim: 300611 Human

SwissProt: Q96EU7 Human

Unigene: 643920 Human



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