Cat. No.
MABL-3503
Application
ELISA, IF
Isotype
Engineer antibody
Species Reactivity
Human
Clone No.
PVA224
From
Recombinant Antibody
Specificity
This antibody specifically recognizes a conformational epitope in the EC1 domain of human desmoglein-3 (DSG) and DSG- This antibody was isolated as a human IgG1 from a patient with mucocutaneous pemphigus vulgaris and uses the VH3-48*03, D4-4*01, JH6*02, VL2-28*01and JL2*02. This antibody has in vitro and in vivo pathogenic activity, as assessed in a keratinocyte dissociation assay and by the formation of microscopic blisters in newborn mice. This antibody crossreacts with murine DSG-3.
Alternative Names
Pemphigus Vulgaris Ag; 130 kDa pemphigus vulgaris antigen; PVA; Cadherin family member 6; DSG3; CDHF6
UniProt
P32926
Immunogen
Made by immortalizing IgG-expressing B cells from Pemphigus vulgaris (PV) patients.
Application Notes
Antibody First Published
Di Zenzo,G. et al. Pemphigus autoantibodies generated through somatic mutations target the desmoglein-3 cis-interface. J Clin Invest 122, 3781–3790 (2012). PMID:22996451
Note on publication
Describes the isolation and analysis of a panel of human antibodies from patients - including data on ELISA, IF and in vitro and in vivo pathogenic activity assays.
Size
100 μg Purified antibody.
Concentration
1 mg/ml.
Purification
Protein A affinity purified
Buffer
PBS with 0.02% Proclin 300.
Storage Recommendation
Store at 4⁰C for up to 3 months. For longer storage, aliquot and store at - 20⁰C.
