This gene encodes a member of the immunoglobulin gene superfamily. Expression of this cell surface protein is restricted to B cell lymphocytes. This protein is a reliable marker for pre-B cells but its expression diminishes during terminal B cell differentiation in antibody secreting plasma cells. The protein has two N-terminal extracellular Ig-like domains separated by a non-Ig-like domain, a hydrophobic transmembrane domain, and a large C-terminal cytoplasmic domain. This protein forms a complex with several membrane proteins including complement receptor type 2 (CD21) and tetraspanin (CD81) and this complex reduces the threshold for antigen-initiated B cell activation. Activation of this B-cell antigen receptor complex activates the phosphatidylinositol 3-kinase signalling pathway and the subsequent release of intracellular stores of calcium ions. This protein is a target of chimeric antigen receptor (CAR) T-cells used in the treatment of lymphoblastic leukemia. Mutations in this gene are associated with the disease common variable immunodeficiency 3 (CVID3) which results in a failure of B-cell differentiation and impaired secretion of immunoglobulins. CVID3 is characterized by hypogammaglobulinemia, an inability to mount an antibody response to antigen, and recurrent bacterial infections. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jul 2020]
产品应用
应用
已检合格种属
预测种属
推荐稀释比例
WB
Human
1:1000-5000
IHC-P
Human
1:200-500
IHC-F
Human
1:200-500
IF
Human
1:200-500
Flow-Cyt
Human
1:50-100
ICC/IF
Human
1:50-200
交叉反应
交叉反应: Human
相关产品
暂无相关产品
靶标
基因名
CD19
蛋白名
B-lymphocyte antigen CD19
亚基
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.
亚细胞定位
Membrane; Single-pass type I membrane protein.
翻译后修饰
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.
疾病
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.
相似性
Belongs to the selectin/LECAM family.
Contains 2 Ig-like C2-type (immunoglobulin-like) domains.
功能
Assembles with the antigen receptor of B-lymphocytes in order to decrease the threshold for antigen receptor-dependent stimulation.