Human Collagen Type VI Alpha 1 ELISA Kit | COL6alpha1 elisa kit

Human Collagen Type VI Alpha 1 ELISA Kit

Cat. Num. AAA762357

• Gene Names: COL6A1; OPLL; BTHLM1; UCHMD1
• Reactivity: Human
• Synonyms: Collagen Type VI Alpha 1; Human Collagen Type VI Alpha 1 ELISA Kit; COL6alpha1/COL6A1/OPL; COL6alpha1 elisa kit

• Reactivity: Human
• Sequence Length: 1028

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 0.156-10ng/ml
• Sensitivity: 0.094ng/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data

Typical Standard Curve

Related Product Information for COL6alpha1 elisa kit

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. anti-Human COL6alpha1 antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human COL6alpha1 antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Human COL6alpha1 amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Human COL6alpha1 can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 119629727
• NCBI GeneID: 1291
• Molecular Weight: 108,529 Da
• NCBI Official Full Name: collagen, type VI, alpha 1, isoform CRA_b, partial
• NCBI Official Synonym Full Names: collagen type VI alpha 1
• NCBI Official Symbol: COL6A1
• NCBI Official Synonym Symbols: OPLL; BTHLM1; UCHMD1
• NCBI Protein Information: collagen alpha-1(VI) chain
• UniProt Protein Name: Collagen alpha-1(VI) chain
• UniProt Gene Name: COL6A1
• UniProt Entry Name: CO6A1_HUMAN

NCBI Description

The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy. [provided by RefSeq, Jul 2008]

Uniprot Description

COL6A1: Collagen VI acts as a cell-binding protein. Defects in COL6A1 are a cause of Bethlem myopathy (BM). BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A1 are a cause of Ullrich congenital muscular dystrophy (UCMD); also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy. Belongs to the type VI collagen family.

Protein type: Secreted; Secreted, signal peptide; Extracellular matrix

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: collagen type VI; endoplasmic reticulum lumen; extracellular matrix; extracellular region; lysosomal membrane; membrane; protein complex; sarcolemma

Molecular Function: platelet-derived growth factor binding

Biological Process: axon guidance; cell adhesion; collagen catabolic process; extracellular matrix disassembly; extracellular matrix organization and biogenesis; osteoblast differentiation

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy