Defects in TNFRSF13C are the cause of immunodeficiency common variable type 4 (CVID4); also called antibody deficiency due to BAFFR defect. CVID4 is a primary immunodeficiency characterized by antibody deficiency, hypogammaglobulinemia, recurrent bacterial infections and an inability to mount an antibody response to antigen. The defect results from a failure of B-cell differentiation and impaired secretion of immunoglobulins; the numbers of circulating B cells is usually in the normal range, but can be low.
Formulation
1 mg/ml in 1X PBS; BSA free, sodium azide free
Host
Mouse
Immunogen Region
Recombinant full-length human CD268 protein was used as the immunogen for the BAFF-R antibody.
Isotype
Mouse IgG1, kappa
Species Reactivity
Human
Note
Optimal dilution of the BAFF-R antibody should be determined by the researcher.
Uniprot
Q96RJ3
Format
Purified
Purity
Protein G affinity chromatography
Storage
Store the BAFF-R antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Applications
IHC-P
Description
Immunohistochemistry (FFPE): 1-2ug/ml for 30 minutes at RT