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  4. Human Collagen Type Ⅵ(COLⅥ) ELISA Kit

Human Collagen Type Ⅵ(COLⅥ) ELISA Kit

  • 中文名称：
    人Ⅵ型胶原(COLⅥ)酶联免疫试剂盒
  • 货号：
    CSB-E16375h
  • 规格：
    96T/48T
  • 价格：
    ￥3600/￥2500
  • 其他：

产品详情

  • 产品描述：

    This Human COL6A1 ELISA Kit was designed for the quantitative measurement of Human COL6A1 protein in serum, plasma, cell culture supernates, urine. It is a Sandwich ELISA kit, its detection range is 31.25 pg/mL-2000 pg/mL and the sensitivity is 7.81 pg/mL .

  • 别名：
    Alpha 1 (VI) chain (61 AA) ELISA Kit; CO6A1_HUMAN ELISA Kit; COL6A1 ELISA Kit; COL6A2 ELISA Kit; COL6A3 ELISA Kit; collagen 6 ELISA Kit; Collagen alpha 2(VI) chain ELISA Kit; Collagen alpha 3(VI) chain ELISA Kit; Collagen alpha-1(VI) chain ELISA Kit; collagen six ELISA Kit; Collagen type VI alpha 1 ELISA Kit; Collagen type VI alpha 2 ELISA Kit; Collagen type VI alpha 3 ELISA Kit; Collagen VI alpha 1 polypeptide ELISA Kit; Collagen VI alpha 2 polypeptide ELISA Kit; Collagen VI alpha 3 polypeptide ELISA Kit; CollagenVI ELISA Kit; Human mRNA for collagen VI alpha 2 C terminal globular domain ELISA Kit; OPLL ELISA Kit; PP3610 ELISA Kit
  • 缩写：
  • Uniprot No.：
  • 种属：
    Homo sapiens (Human)
  • 样本类型：
    serum, plasma, cell culture supernates, urine
  • 检测范围：
    31.25 pg/mL-2000 pg/mL
  • 灵敏度：
    7.81 pg/mL
  • 反应时间：
    1-5h
  • 样本体积：
    50-100ul
  • 检测波长：
    450 nm
  • 研究领域：
    Signal Transduction
  • 测定原理：
    quantitative
  • 测定方法：
    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.
  • 线性度：
    To assess the linearity of the assay, samples were spiked with high concentrations of human COLVI in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    　SampleSerum(n=4)
    1:200Average %86
    Range %80-96
    1:400Average %97
    Range %91-98
    1:800Average %98
    Range %92-105
    1:1600Average %102
    Range %86-110
  • 回收率：
    The recovery ofhuman COLVI 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 TypeAverage % RecoveryRange
    Serum (n=5) 9889-110
    EDTA plasma (n=4)9690-100
  • 标准曲线：
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    pg/mlOD1OD2AverageCorrected
    20002.912 2.812 2.862 2.702
    10002.137 2.103 2.120 1.960
    5001.156 1.098 1.127 0.967
    2500.614 0.578 0.596 0.436
    1250.372 0.346 0.359 0.199
    62.50.281 0.279 0.280 0.120
    31.250.202 0.198 0.200 0.040
    00.161 0.159 0.160 　
  • 数据处理：
  • 货期：
    3-5 working days

产品评价

靶点详情

  • 功能：
    Collagen VI acts as a cell-binding protein.
  • 基因功能参考文献：
    1. FKBP10 interacts with collagen VI and deficiency of FKBP10 reduces lung fibroblast migration by down-regulation of collagen VI synthesis. PMID: 29673351
    2. Five SNPs in the COL6A1 (and IL17RC) genes were found to be associated with susceptibility to ossification of the posterior longitudinal ligament in Han Chinese patients. PMID: 29764467
    3. the donor splice site of COL6A1 intron 14, associated with the phenotype of Bethlem myopathy or intermediate form, is a hot spot for ColVI myopathies PMID: 28984114
    4. The polypeptide is a novel non-triple helical polypeptide of type VI collagen alpha1 chain encoded by COL6A1, or NTH alpha1(VI). PMID: 29659864
    5. Missense mutations in COL6A1, COL11A2, FGFR1, and BMP2 genetically predispose patients to ossification of posterior longitudinal ligaments. PMID: 27246988
    6. We have used RNA-Seq to identify differentially expressed genes in cultured dermal fibroblasts from 13 COL6-RD individuals (8 dominant negative and 5 null) and 6 controls. To better assess the transcriptional changes induced by abnormal collagen VI in the extracellular matrix (ECM); we compared transcriptional profiles from subjects with DN mutations and subjects with null mutations to transcriptional profiles PMID: 29244830
    7. These data demonstrate, for the first time, a functional relationship between collagens VI and XII during osteogenesis. PMID: 26753503
    8. COL6A1 may have a role in progression and outcome of clear cell renal cell carcinoma PMID: 26317545
    9. upregulated in the airways of chronic obstructive pulmonary disease patients and exposed upon epithelial desquamation PMID: 25925694
    10. is the first report of UCMD recurrence in 2 siblings due to a germline mosaic COL6 gene mutation PMID: 25978941
    11. worsening of the functional disability appeared typically after the age of 40 in 47% of our patients with Bethlem myopathy, and was frequently associated with COL6A1 exon 14 skipping PMID: 25535305
    12. The second main finding of this study was that COL6A1 rs35796750 did not associate with the risk of anterior cruciate ligament injury in the self-reported Caucasian South African cohort. PMID: 25073002
    13. Type VI collagen and activated retinal Muller cells are present in iERM. PMID: 26447986
    14. Data indicate that collagen-VI-alpha-1 (COL6A1) is expressed in all grades of glioma. PMID: 25325876
    15. Data suggest the potential role of COL6 in promoting lung neoplasia in diseased lungs where COL6 is overexpressed. PMID: 25176343
    16. Parental mosaicism was confirmed in the four families through quantitative analysis of the ratio of mutant versus wild-type allele (COL6A1, COL6A2, and COL6A3) in genomic DNA from various tissues; consistent with somatic mosaicism, parental samples had lower ratios of mutant versus wild-type allele compared with the fully heterozygote offspring. PMID: 25204870
    17. In UCDM, 1 mutation was indentified in COL6A1 in Chinese patients. PMID: 24801232
    18. These results suggest that these SNPs of BMP-2 and COL6A1 may not directly influence the expression of OPLL. PMID: 24737472
    19. Absence of ANXA2 leads to retention of COL6 in a late-Golgi, VAMP2-positive compartment. PMID: 24357721
    20. Mutations in each of the three collagen VI genes, COL6A1, COL6A2 and COL6A3, cause four types of muscle disorders: Ullrich congenital muscular dystrophy, Bethlem myopathy, limb-girdle muscular dystrophy, and autosomal recessive myosclerosis. (Review) PMID: 24443028
    21. The resulting proposed clinical classification system of collagen VI-related myopathy is unique in that it is based on the integration of both motor function and pulmonary function criteria. PMID: 24271325
    22. XPD mutations in trichothiodystrophy hamper COL6A1 expression. PMID: 23221806
    23. TP-alpha, collagen alpha-1(VI) chain and S100A9 are potential biomarkers of esophageal squamous cell carcinoma, and may play an important role in tumorigenesis and development of ESCC. PMID: 22583932
    24. the COL6A1 rs35796750 TT genotype is associated with increased performance during the bicycling of the South African Ironman triathlon PMID: 22012643
    25. the accumulation of abnormal mitochondria and sarcoplasmic reticulum is caused by a defect of autophagy and that restoration of a proper autophagic flux in Col6a1-/- muscles ameliorates these alterations. PMID: 21037586
    26. This study revealed several genotype-phenotype correlations, providing new insights into the natural history and course of ColVI myopathies. PMID: 20976770
    27. COL6 genes encoding type VI collagen PMID: 11932968
    28. Haplotype analysis clearly suggested linkage of Ullrich muscular dystrophy to the COL6A1/2 locus in two cases and to the COL6A3 loci in the third case. In the remaining nine patients, primary collagen VI involvement was excluded PMID: 12011280
    29. Bethlem myopathy is an autosomal dominantly inherited myopathy with contractures caused by mutations in the COL6A1 gene. PMID: 12374585
    30. Collagen VI deficiency might have caused electron microscopic changes of capillaries, while function of capillaries is apparently retained. PMID: 12736748
    31. a de novo heterozygous deletion of the COL6A1 gene results in a severe phenotype of classical Ullrich congenital muscular dystrophy PMID: 12840783
    32. linkage disequilibrium and association studies that SNPs in the collagen 6A1 gene (COL6A1) were strongly associated with Ossification of the posterior longitudinal ligament PMID: 12958705
    33. The failure of collagen VI to anchor the basal lamina to the interstitium is the cause of Ullrich disease. PMID: 14981181
    34. dominant mutations are common in Ullrich congenital muscular dystrophy (UCMD). PMID: 15563506
    35. we report a genotype-phenotype correlation demonstrating that heterozygous glycine substitutions in the triple-helix domain of COL6A1 are dominant and responsible for a milder Ullrich scleroatonic muscular dystrophy phenotype. PMID: 16130093
    36. COL6A1 could be responsible for the hyperostotic state, leading to ectopic bone formation in the spinal ligament. PMID: 16227896
    37. beta ig-h3 can differentially modulate the aggregation of collagen VI with biglycan and decorin PMID: 16434404
    38. Major promoter and enhancer sequences regulating COL6A1 expression are present in this bacterial artificial chromosome clone. PMID: 17334655
    39. This study identified a novel homozygous COL6A1 premature termination mutation in a UCMD patient that causes nonsense-mediated mRNA decay. PMID: 17537636
    40. This study demonstrates a homogeneous overexpression of the genes encoding for alpha1 and alpha2 chains for collagen type VI in nuchal skin of human trisomy 21 fetuses. PMID: 17602442
    41. COL6A1 may be a common susceptibility gene for ossification of the ligamentum flavum and ossification of the posterior longitudinal ligament in Chinese Han population. PMID: 18246005
    42. Study reports 10 unrelated patients with a Ullrich congenital muscular dystrophy clinical phenotype and de novo dominant negative heterozygous splice mutations in COL6A1, COL6A2 and COL6A3. PMID: 18366090
    43. Immunofluorescent labeling of collagen VI in fibroblast cultures is a useful addition to current diagnostic services for Bethlem myopathy (BM). It can be used to guide molecular genetic testing in a cost-effective and time-saving manner. PMID: 18378883
    44. Results found COL6A1 to be differentially expressed in human astrocytomas. PMID: 18551403
    45. SNP of COL6A1 were not related to radiographic progression of ankylosing spondylitis. PMID: 18634150
    46. These data indicate that collagen VI glycine mutations impair the assembly pathway in different ways and disease severity correlates with the assembly abnormality. PMID: 18825676
    47. Four patients affected by Ullrich congenital muscular dystrophy and carrying unusual mutations of COL6 genes affecting RNA splicing, were identified. PMID: 19309692

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  • 相关疾病：
    Bethlem myopathy 1 (BTHLM1); Ullrich congenital muscular dystrophy 1 (UCMD1)
  • 亚细胞定位：
    Secreted, extracellular space, extracellular matrix.
  • 蛋白家族：
    Type VI collagen family
  • 数据库链接：

    HGNC: 2211

    OMIM: 120220

    KEGG: hsa:1291

    STRING: 9606.ENSP00000355180

    UniGene: Hs.474053