• Recombinant Human BP180/NC16a Protein
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AntibodySystem

AID00701

Recombinant Human BP180/NC16a Protein

Recombinant Human BP180/NC16a Protein

Catalog No.: AID00701

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.

Catalog No. AID00701
Product name Recombinant Human BP180/NC16a Protein
Size 100 μg
Expression system Confidential
Protein length Confidential
Accession Q9UMD9
Description 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.
Form Lyophilized
Storage buffer Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Purity >95% as determined by SDS-PAGE.
Applications As a diagnostic tool for bullous pemphigoid.
Endotoxin level Please contact with the lab for this information.
Nature Recombinant
Stability and Storage 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.
Reconstitution Reconstitute in sterile water for a stock solution.
Alternative Names 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
Species Homo sapiens (Human)
Shipping In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Note For research use only.
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