Antibody deficiency due to defect in CD19, included; AW495831; B lymphocyte antigen CD19; B lymphocyte surface antigen B4; B4; CD 19; CD19 antigen; CD19 molecule; Cd19 protein; Differentiation Antigen CD19; Leu 12; Leu12; Lymphocyte Surface Antigen; MGC10
Cat:
SLM30096M-FITC
Species Reactivity：
Human,
Immunogen：
KLH conjugated synthetic peptide derived from human CD19
Format：
Lyophilized or Liquid
Storage instructions：
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer：
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration：
1mg/ml
Clonality：
Monoclonal
Isotype：
IgG
Applications：
Flow-Cyt=5ul/TestICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host：
Mouse
Calculated MW：
59kDa
Product Overview：
scatter diagram showing peripheral blood lymphocytes stained with SLM30096M-FITC. The cells were incubated with the antibody (SLM30096M-FITC, 5 µl/Test) for 30 min at 22°C. Acquisition of >10,000 events was performed.
background: CD19 is a transmembrane glycoprotein that is delectively expressed on the cell surface of B-lymphocytes,where it activates intracellular signaling cascades involving both Ras and phosphatidylinositol 3-kinase pathways.Lymphocytes proliferate and differentiate in response to various concentrations of different antigens. The ability of the B cell to respond in a specific, yet sensitive manner to the various antigens is achieved with the use of low-affinity antigen receptors. This gene encodes a cell surface molecule which assembles with the antigen receptor of B lymphocytes in order to decrease the threshold for antigen receptor-dependent stimulation.
Function: Assembles with the antigen receptor of B-lymphocytes in order to decrease the threshold for antigen receptor-dependent stimulation.
Subunit: Forms a complex with CD21, CD81 and CD225 in the membrane of mature B-cells. Interacts with VAV. Interacts with GRB2 and SOS when phosphorylated on Tyr-348 and/or Tyr-378. Interacts with PLCG2 when phosphorylated on Tyr-409. Interacts with LYN.
Subcellular Location: Membrane; Single-pass type I membrane protein.
Post-translational modifications: Phosphorylated on serine and threonine upon DNA damage, probably by ATM or ATR. Phosphorylated on tyrosine following B-cell activation. Phosphorylated on tyrosine residues by LYN.
DISEASE: Defects in CD19 are the cause of immunodeficiency common variable type 3 (CVID3) [MIM:613493]; also called antibody deficiency due to CD19 defect. CVID3 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.
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Picture
scatter diagram showing peripheral blood lymphocytes stained with SLM30096M-FITC. The cells were incubated with the antibody (SLM30096M-FITC, 5 µl/Test) for 30 min at 22°C. Acquisition of >10,000 events was performed.
