Catalog No.
AID00701
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.
Expression system
Confidential
Species
Homo sapiens (Human)
Protein length
Confidential
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>95% as determined by SDS-PAGE.
Accession
Applications
As a diagnostic tool for bullous pemphigoid.
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
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.
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
For research use only. Not for human or drug use.
