Recombinant Human BP180/NC16a Protein

Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells) . The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin) . These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters.

Product Specifications

Product Name Alternative

Linear IgA disease antigen 1, COL17A1, 180 kDa bullous pemphigoid antigen 2, LAD-1, Linear IgA bullous disease antigen of 97 kDa, 120 kDa linear IgA dermatosis antigen, 97-LAD, Collagen alpha-1 (XVII) chain, Bullous pemphigoid antigen 2, 97 kDa linear IgA bullous dermatosis antigen, LABD97, 97 kDa LAD antigen, BPAG2, BP180

Accession Number

Q9UMD9

Expression System

Confidential

Nature

Recombinant

Applications

As a diagnostic tool for bullous pemphigoid.

Stability

Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.