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

Code CSB-EL005753HU
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Product Type ELISA Kit
Size 96T,5×96T,10×96T
Uniprot No. P12111
Lead Time 5-7 working days
Abbreviation COL6A3
Protein Biological Process 1 Cell Adhesion
Target Name collagen, type VI, alpha 3
Alias DKFZp686D23123, DKFZp686K04147, DKFZp686N0262, FLJ34702, FLJ98399, OTTHUMP00000202895|alpha 3 type 6 collagen|collagen 6, alpha-3 polypeptide|collagen alpha-3(6) chain,Collagen alpha-3(VI) chain (COL6A3)
Species Homo sapiens (Human)
Protein Biological Process 3 Cell adhesion
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
Protocol
Protocol may be improved. Please feel free to contact CUSABIO product specialist to obtain the latest version.
Assay Principle quantitative
Measurement Sandwich
Target Details This gene encodes the alpha-3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is much larger than the alpha-1 and -2 chains. This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all the alpha chains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in the type VI collagen genes are associated with Bethlem myopathy, a rare autosomal dominant proximal myopathy with early childhood onset. Mutations in this gene are also a cause of Ullrich congenital muscular dystrophy, also referred to as Ullrich scleroatonic muscular dystrophy, an autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Multiple transcript variants have been identified, but the full-length nature of only some of these variants has been described.
HGNC 2213
MGI 88461
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      
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Function Collagen VI acts as a cell-binding protein.
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

Pathway PI3K-Akt signaling pathway
Focal adhesion

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