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C1GALT1C1 Antibody

  • 货号：
    CSB-PA003490LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunofluorescence staining of U251 cells with CSB-PA003490LA01HU at 1:100, counter-stained with DAPI. The cells were blocked in 10% normal Goat Serum and incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) C1GALT1C1 Polyclonal antibody
  • Uniprot No.：
    Q96EU7
  • 基因名：
    C1GALT1C1
  • 别名：
    C1GALT1-specific chaperone 1 (C38H2-like protein 1) (C38H2-L1) (Core 1 beta1,3-galactosyltransferase 2) (C1Gal-T2) (C1GalT2) (Core 1 beta3-Gal-T2) (Core 1 beta3-galactosyltransferase-specific molecular chaperone), C1GALT1C1, COSMC
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human C1GALT1-specific chaperone 1 protein (27-318aa)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，C1GALT1C1 Antibody (CSB-PA003490LA01HU)，的标记方式是Non-conjugated。对于C1GALT1C1 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA003490LB01HU C1GALT1C1 Antibody, HRP conjugated ELISA
    FITC CSB-PA003490LC01HU C1GALT1C1 Antibody, FITC conjugated
    Biotin CSB-PA003490LD01HU C1GALT1C1 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IF 1:100-1:500
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    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).
  • 基因功能参考文献：
    1. In addition, we found that Tn-expressing colorectal cancers (CRC) cell lines had either loss-of-function mutations in Cosmc or reversible Tn antigen expression, which was not caused by the deficiency of T-synthase activity PMID: 30115016
    2. elevated Tn levels in cancer and inflammation may be commonly regulated by the cytokine (TNFa and IL6)-Cosmc signaling axis PMID: 27542280
    3. The data suggest that hypermethylation of the Cosmc promoter may induce the expression of Tn antigen in activated T cells. PMID: 28708980
    4. results provide new structure-function insight to Cosmc, indicate that Cosmc behaves as a modular protein and suggests points of modulation or regulation of in vivo chaperone function PMID: 28665962
    5. Data indicate that mRNA levels of both core 1 synthase, glycoprotein-N-acetylgalactosamine 3-beta-galactosyltransferase, 1 (C1GALT1) and C1GALT1-specific chaperone 1 protein (C1GALT1C1) determine the rate of secretion of galactose-deficient IgA1 (Gd-IgA1) in IgA1-producing cells. PMID: 28187132
    6. The terminal glycosylation of secreted IgA1 was altered in response to TGFbeta1. TGFbeta1 stimulation significantly decreased the mRNA levels of C1GalT1 and Cosmc. TGFbeta1 may be key in controlling the glycosylation of IgA1, in part via the downregulation of C1GalT1 and Cosmc. PMID: 28035353
    7. mutations c.393T>A and c.72A>G in the exon region of Cosmc gene in children with Henoch-Schonlein purpura (HSP) are not associated with the onset of Henoch-Schonlein purpura PMID: 27412546
    8. Data suggest that patients with IgA nephropathy exhibit higher microRNA-374b in B cells compared to controls; microRNA-374b appears to target PTEN (phosphatase and tensin homolog) and Cosmc (C1GALT1 specific chaperone 1) proteins. PMID: 26545495
    9. These results indicate that Tn antigens expression and T-synthase inactivity in HT-29-Tn+ cells can be related to the absence of the Cosmc gene coding sequence in Cosmc active alleles PMID: 26045765
    10. the impact of COSMC mediated Tn antigen expression in two human pancreatic ductal adenocarcinoma cell lines on cellular oncogenic properties, were investigated. PMID: 26021314
    11. hypermethylation of Cosmc promoter region could be a key mechanism for the reduction of Cosmc mRNA expression in IgAN lymphocytes with associated increase in aberrantly glycosylated IgA1 PMID: 25647400
    12. Cosmc and T-synthase are transcriptionally regulated at a basal level by the specificity protein/Kruppel-like transcription factor family of members. PMID: 26063800
    13. CBRT is a unique recognition motif for Cosmc to promote its regulation and formation of active T-synthase and represents the first sequence-specific chaperone recognition system in the ER/Golgi required for normal protein O-glycosylation PMID: 24616093
    14. Data indicate that cytotoxin associated gene A protein (CagA) promoted the underglycosylation of IgA1, which at least partly attributed to the downregulation of beta1,3-galactosyltransferase (C1GALT1) and its chaperone Cosmc. PMID: 24462875
    15. overexpression of Cosmc is associated with colorectal cancer. PMID: 23390052
    16. COSMC is a novel regulator for VEGFR2 signaling in endothelial cells and dysregulation of COSMC expression may contribute to the pathogenesis of hemangioma PMID: 23424651
    17. The mRNA expression level of Cosmc gene in IgA nephropathy patients was significantly lower than that of controls. De-methylation modification up regulated the Cosmc gene expression significantly. PMID: 22332537
    18. Results show that soluble Cosmc directly interacts in a specific manner with denatured, but not native, T-synthase to form a noncovalent and reversible complex that results in the acquisition of T-synthase catalytic activity. PMID: 22416136
    19. Results indicate that Cosmc mediates the co-translational activation of C1GalT and that it may prevent the unfavorable aggregation of C1GalT. PMID: 21496458
    20. The transmembrane domain of the molecular chaperone Cosmc directs its localization to the endoplasmic reticulum PMID: 21262965
    21. Study results suggest that C1GALT1C1 may play a key role in the regulation of IgA1 O-glycosylation. PMID: 20144270
    22. Cosmc represents the first endoplasmic reticulum chaperone identified to be required for folding of a glycosyltransferase PMID: 19923218
    23. molecular cloning and characterization; C1Gal-T2 is the second candidate for core 1 synthase that plays an important role in synthesizing O-glycans in digestive organs PMID: 12361956
    24. Tn syndrome is associated with a somatic mutation in Cosmc, a gene on the X chromosome that encodes a molecular 'chaperone' that is required for the proper folding and hence full activity of T-synthase PMID: 16251947
    25. These results suggest that the intracellular dynamics of C1GalT is controlled by its specific molecular chaperon, Cosmc, in association with core 1 synthase activity. PMID: 18061573
    26. The tumor-specific antigen caused by mutant COSMC seems to be rare and is not potentially a therapeutic target candidate in breast and colon cancers. PMID: 18321367
    27. Colon cancer and melanoma-derived cells lines expressed Tn and STn antigen due to loss-of-function mutations in Cosmc. Cervical cancer specimens that showed expression of the Tn/STn antigens were also found to have mutations in Cosmc. PMID: 18339842
    28. a novel inactivating mutations (Glu152Lys, Ser193Pro and Met1Ile) in the coding sequence of C1GALT1C1 PMID: 18537974
    29. Cosmc is an endoplasmic reticulum (ER)-localized adenosine triphosphate binding chaperone that binds directly to human T-synthase. PMID: 18695044
    30. Although decreased C1GALT1 activity has been implicated in IgAN pathogenesis and cosmc chaperone mutations can cause autoimmune disease, our data provide no evidence for a role of cosmc gene mutations in European patients with sporadic or familial IgAN. PMID: 18840896
    31. The c.-347-190G>A polymorphism and the somatic mutation of encoding region of C1GALT1C1 gene were not significantly related to the genetic susceptibility to IgAN in Northern Chinese population PMID: 19778426

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  • 相关疾病：
    Tn polyagglutination syndrome (TNPS)
  • 亚细胞定位：
    Membrane; Single-pass type II membrane protein.
  • 蛋白家族：
    Glycosyltransferase 31 family, Beta3-Gal-T subfamily
  • 组织特异性：
    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.
  • 数据库链接：

    HGNC: 24338

    OMIM: 300611

    KEGG: hsa:29071

    STRING: 9606.ENSP00000304364

    UniGene: Hs.643920