AID00701
Recombinant Human BP180/NC16a Protein
Size
£168.00
- SKU:
- AID00701
- Additional Names:
- LinearIgAdiseaseantigen1,COL17A1,180kDabullouspemphigoidantigen2,LAD-1,LinearIgAbullousdiseaseantigenof97kDa,120kDalinearIgAdermatosisantigen,97-LAD,Collagenalpha-1(XVII)chain,Bullouspemphigoidantigen2,97kDalinearIgAbullousdermatosisantigen,LABD97,97kDaLADantigen,BPAG2,BP180
- Physical State:
- Lyophilized
- Purity:
- >95% as determined by sds-page.
- Storage Conditions:
- -70[o]C Avoid freeze/thaw cycles., 2-8[o]C Avoid freeze/thaw cycles.
- Supplier:
- AntibodySystems
- Buffer:
- LyophilizedfromasolutioninPBSpH7.4,0.02%NLS,1mMEDTA,4%Trehalose,1%Mannitol.
- Species:
- Human
- Uniprot:
- Q9UMD9
- Synonyms:
- 180 kDa bullous pemphigoid antigen 2;alpha 1 type XVII collagen;BA16H23.2;bA16H23.2 (collagen, type XVII, alpha 1 (BP180));BP180;BPA-2;BPAG2;Bullous pemphigoid antigen 2;bullous pemphigoid antigen 2 (180kD);collagen alpha-1(XVII) chain;collagen XVII, alpha-1 polypeptide;collagen, type XVII, alpha 1;ERED;LAD-1;type XVII collagen alpha-1
- Extra Details:
- BullouspemphigoidistheresultofanattackonthebasementmembraneoftheepidermisbyIgG+/-IgEimmunoglobulins(antibodies)andactivatedTlymphocytes(whitebloodcells).ThetargetistheproteinBP180(alsocalledTypeXVIIcollagen),orlessfrequentlyBP230(aplakin).TheseproteinsarewithintheNC16AdomainofcollagenXVII.Theyareassociatedwiththehemidesmosomes,structuresthatensuretheepidermalkeratinocytecellssticktothedermistomakeawaterproofseal.ThebindingoftheautoantibodiestotheproteinsandreleaseofcytokinesfromtheTcellsleadtocomplementactivation,recruitmentofneutrophils(acuteinflammatorycells)andthereleaseofproteolyticenzymes.Thesedestroythehemidesmosomesandcausetheformationofsubepidermalblisters.
- Shipping Conditions:
- Dry Ice