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  4. Human Collagen alpha-3(VI) chain (COL6A3) ELISA kit

Human Collagen alpha-3(VI) chain (COL6A3) ELISA kit

  • 中文名稱：
    人VI型膠原α-3鏈(COL6A3)酶聯免疫試劑盒
  • 貨號：
    CSB-EL005753HU
  • 規(guī)格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產品詳情

  • 產品描述：
    人VI型膠原α-3鏈(COL6A3)酶聯免疫試劑盒（CSB-EL005753HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿樣本中的COL6A3含量。人VI型膠原α-3鏈（COL6A3）是編碼VI型膠原蛋白α-3鏈的基因，存在于大多數結締組織中，其突變與多種肌肉疾病相關，如貝特萊姆肌病和烏爾里希先天性肌營養(yǎng)不良癥。此外，COL6A3在結直腸癌、胰腺癌、卵巢癌和胃癌中表達升高，可能與腫瘤的發(fā)生發(fā)展有關。試劑盒檢測范圍為1.56 ng/mL-100 ng/mL，適用于多種生物樣本中COL6A3的定量分析，尤其適合研究肌肉組織修復機制、纖維化疾病模型或細胞外基質動態(tài)變化等科研場景。本試劑盒為研究COL6A3在組織工程、疾病模型構建及分子機制探索中的應用提供可靠工具。本品僅用于科研，不用于臨床診斷，產品具體參數及操作步驟詳見產品說明書。
  • 別名：
    CO6A3_HUMAN ELISA Kit; COL6A3 ELISA Kit; Collagen alpha-3(VI) chain ELISA Kit; Collagen type VI alpha 3 ELISA Kit; Collagen VI alpha 3 ELISA Kit
  • 縮寫：
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    serum, plasma, tissue homogenates
  • 檢測范圍：
    1.56 ng/mL-100 ng/mL
  • 靈敏度：
    0.39 ng/mL
  • 反應時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領域：
    Signal Transduction
  • 測定原理：
    quantitative
  • 測定方法：
    Sandwich
  • 精密度：
    Intra-assay Precision (Precision within an assay): CV%<8%
    Three samples of known concentration were tested twenty times on one plate to assess.
    Inter-assay Precision (Precision between assays): CV%<10%
    Three samples of known concentration were tested in twenty assays to assess.
  • 線性度：
    To assess the linearity of the assay, samples were spiked with high concentrations of human COL6A3 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    　 Sample Serum(n=4)
    1:1 Average % 86
    Range % 80-89
    1:2 Average % 91
    Range % 87-95
    1:4 Average % 92
    Range % 87-98
    1:8 Average % 93
    Range % 86-97
  • 回收率：
    The recovery of human COL6A3 spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
    Sample Type Average % Recovery Range
    Serum (n=5) 87 82-91
    EDTA plasma (n=4) 102 97-106
  • 標準曲線：
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    ng/ml OD1 OD2 Average Corrected
    100 1.988 1.872 1.930 1.821
    50 1.192 1.262 1.227 1.118
    25 0.724 0.769 0.747 0.638
    12.5 0.451 0.467 0.459 0.350
    6.25 0.304 0.314 0.309 0.200
    3.12 0.217 0.222 0.220 0.111
    1.56 0.160 0.169 0.165 0.056
    0 0.108 0.109 0.109 　
  • 數據處理：
  • 貨期：
    3-5 working days

產品評價

靶點詳情

  • 功能：
    Collagen VI acts as a cell-binding protein.
  • 基因功能參考文獻：
    1. COL6A3 could influence the viability and angiogenesis of bladder cancer cells. COL6A3 may have a certain relationship with the TGF-beta/Smad-induced EMT process. PMID: 30066698
    2. The morphology and immunophenotype of all 6 cases was analogous to those with the canonical COL1A1-PDGFB fusion; none of the cases showed fibrosarcomatous transformation. This study illustrates that the COL6A3-PDGFD fusion product is rare in dermatofibrosarcoma protuberans, and associated with an apparent predilection for breast PMID: 30014607
    3. Study found COL6A3 expression to be downregulated and associated with poor prognosis in human colorectal cancer (CRC). In silico analysis of cell typespecific gene expression and COL6A3 knockout experiments indicated the clinical relevance of COL6A3 in the development of CRC. PMID: 29620224
    4. COL6A mutation Congenital Muscular Dystrophy showed the muscle weakness and poor respiratory function. PMID: 29465610
    5. two compound heterozygous mutations in COL6A3 gene lead to myopathy from Ullrich congenital muscular dystrophy and Bethlem myopathy spectrum. PMID: 29894794
    6. COL6A3-associated dystonia represents a newly identified autosomal-recessive entity characterized clinically by an early symptom onset with variable distribution. PMID: 26687111
    7. Overexpression of endotrophin led to a fibrotic program in white adipose tissue (WAT) adipocytes, a proinflammatory program in (WAT) macrophages, and upregulation of both profibrotic and proinflammatory genes in the stromal vascular fraction isolated from WAT. PMID: 27729337
    8. Patients with chronic kidney disease (CKD) are at increased risk of end-stage renal disease (ESRD) and early mortality. Serum endotrophin, a COL6A3 cleavage product was significantly associated with progression to ESRD. PMID: 28403201
    9. In conjunction with the relatively high frequency of homozygous carriers of reported mutations in publically available databases, our data call a causal role for variants in COL6A3 in isolated dystonia into question. PMID: 26872670
    10. Data indicate that circulating plasma COL6A3 in colorectal cancer (CRC) patients was upregulated significantly comparing with healthy peoples. PMID: 26338966
    11. COL6A mutations were identified in eight cases having clinical phenotypes of Ullrich congenital muscular dystrophy (UCMD) or Bethlem myopathy (BM). PMID: 25635128
    12. Recessive mutations in the alpha3 (VI) collagen gene COL6A3 cause early-onset isolated dystonia. PMID: 26004199
    13. Increased adipocyte COL6A3 expression associates with insulin resistance; COL6A3 mRNA associates with small adipocyte size PMID: 24719315
    14. The heterozygous c.3353A>C mutation in exon 8 of the COL6A3 gene is associated with the Bethlem myopathy with autosomal dominant inheritance. PMID: 25449070
    15. This study showed that COL6A3 expression appeared to be lowered in obesity, whereas diet- and surgery-induced weight loss increased COL6A3 expression. PMID: 25337653
    16. In UCMD, 1 mutation was indentified in Chinese patients. PMID: 24801232
    17. Data indicate that endotrophin (COL6alpha3) levels are higher in diabetic patients. PMID: 24647224
    18. Postranslational processing of type VI collagen in articular cartilage was investigated: alpha3(VI) collagen C5 domain is initially incorporated into the newly formed type VI fibrils, but after secretion is cut and not in the mature pericellular matrix PMID: 11785962
    19. Mutations in COL6A3 cause severe and mild phenotypes of Ullrich congenital muscular dystrophy. PMID: 11992252
    20. The C-terminal Kunitz-type domain from the alpha3 chain of human type VI collagen (C5), a single amino-acid residue chain with three disulfide bridges, was refined at 0.9 A resolution in a monoclinic form PMID: 12077460
    21. These results suggest that different alpha3(VI) chain isoforms, containing also domains of the N10-N7 region, are required for assembling a proper collagen VI network in the extracellular matrix. PMID: 15965965
    22. the alpha3(VI) C5 domain is present in the extracellular matrix of SaOS-2 N6-C5 expressing cells and fibroblasts, which demonstrates that processing of the C-terminal region of the alpha3(VI) chain is not essential for microfibril formation PMID: 16613849
    23. Col6A3 fusion with colony-stimulating factor-1 gene is associated with tenosynovial giant cell tumors. PMID: 17918257
    24. in humans increased COL6A3 mRNA is associated with adipose tissue macrophage chemotaxis and inflammation and that weight gain PMID: 19837927

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  • 相關疾?。?/div>
    Bethlem myopathy 1 (BTHLM1); Ullrich congenital muscular dystrophy 1 (UCMD1); Dystonia 27 (DYT27)
  • 亞細胞定位：
    Secreted, extracellular space, extracellular matrix.
  • 蛋白家族：
    Type VI collagen family
  • 數據庫鏈接：

    HGNC: 2213

    OMIM: 120250

    KEGG: hsa:1293

    STRING: 9606.ENSP00000295550

    UniGene: Hs.233240