生物
X连锁无丙种球蛋白血症
酪氨酸激酶
酪氨酸
分子生物学
布鲁顿酪氨酸激酶
激酶
细胞生物学
信号转导
生物化学
作者
Satoshi Tsukada,Douglas C. Saffran,David J. Rawlings,Ornella Parolini,R. Cutler Allen,Ivana Klisak,Robert S. Sparkes,Hiromi Kubagawa,T. Mohandas,Shirley G. Quan,John W. Belmont,Max D. Cooper,Mary Ellen Conley,Owen N. Witte
出处
期刊:Cell
[Elsevier]
日期:1993-01-01
卷期号:72 (2): 279-290
被引量:1271
标识
DOI:10.1016/0092-8674(93)90667-f
摘要
We describe a novel cytoplasmic tyrosine kinase, termed BPK (B cell progenitor kinase), which is expressed in all stages of the B lineage and in myeloid cells. BPK has classic SH1, SH2, and SH3 domains, but lacks myristylation signals and a regulatory phosphorylation site corresponding to tyrosine 527 of c-src. BPK has a long, basic amino-terminal region upstream of the SH3 domain. BPK was evaluated as a candidate for human X-linked agammaglobulinemia (XLA), an inherited immunodeficiency characterized by a severe deficit of B and plasma cells and profound hypogammaglobulinemia. BPK mapped to within 100 kb of a probe defining the polymorphism most closely linked to XLA at DXS178. Reduction in or the absence of BPK mRNA, protein expression, and kinase activity was observed in XLA pre-B and B cell lines. BPK is likely the XLA gene and functions in pathways critical to B cell expansion.
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