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

Instructions
Code CSB-EL005753HU
Size 96T,5×96T,10×96T
See More Details 24T ELISA kits trial application
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Product Details

Target Name collagen, type VI, alpha 3
Alternative Names 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
Abbreviation COL6A3
Uniprot No. P12111
Species Homo sapiens (Human)
Sample Types serum, plasma, tissue homogenates
Detection Range 1.56 ng/mL-100 ng/mL
Sensitivity 0.39 ng/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Signal Transduction
Assay Principle quantitative
Measurement Sandwich
Precision
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.      
               
Linearity
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    
Recovery
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    
               
               
Typical Data
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      
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 5-7 working days

Target Data

Function Collagen VI acts as a cell-binding protein.
Gene References into Functions
  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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Involvement in disease Bethlem myopathy 1 (BTHLM1); Ullrich congenital muscular dystrophy 1 (UCMD1); Dystonia 27 (DYT27)
Subcellular Location Secreted, extracellular space, extracellular matrix
Protein Families Type VI collagen family
Database Links

HGNC: 2213

OMIM: 120250

KEGG: hsa:1293

STRING: 9606.ENSP00000295550

UniGene: Hs.233240

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