BCR Antibody – Rabbit Polyclonal

A reciprocal translocation between chromosomes 22 and 9 produces the Philadelphia chromosome, which is often found in patients with chronic myelogenous leukemia. The chromosome 22 breakpoint for this translocation is located within the BCR gene. The translocation produces a fusion protein which is encoded by sequence from both BCR and ABL, the gene at the chromosome 9 breakpoint. Although the BCR-ABL fusion protein has been extensively studied, the function of the normal BCR gene product is not clear. The protein has serine/threonine kinase activity and is a GTPase-activating protein for p21rac. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Bcr: a protein with serine/threonine protein kinase activity and is a GTPase-activating protein (GAP) for RAC1 and CDC42. The amino-terminal region of Bcr contains an oligomerization domain, a serine/threonine kinase domain and a region that binds SH2 domains. The middle of the protein has a PH domain and a region of sequence similarity to the guanine nucleotide exchange factors for the Rho family of GTP binding proteins. The carboxy-terminal region promotes the exchange of RAC or CDC42-bound GDP by GTP, thereby activating them. A breakpoint cluster region protein that participates in a t(9;22)(q34;q11) chromosomal translocation that produces a BCR-ABL oncogene responsible for chronic myeloid leukemia (CML), acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL). The function of wild type Bcr in cells remains unclear. PDGF receptor may use Bcr as a downstream signaling mediator. Tyr177 of human Bcr, phosphorylated in the Bcr-Abl fusion protein, provides a docking site for Gab2 and GRB2, and is important in the transforming activity of Bcr-Abl. Sequence variants of the Bcr protein may be associated with bipolar disorder. Two alternatively spliced isoforms of the human protein have been reported.

Protein type: Kinase, protein; Protein kinase, Ser/Thr (non-receptor); Oncoprotein; EC 2.7.11.1; GEFs, Rac/Rho; GAPs; Protein kinase, atypical; GAPs, Rac/Rho; ATYPICAL group; BCR family

Chromosomal Location of Human Ortholog: 22q11.23

Cellular Component: cell junction; cytosol; membrane; postsynaptic density; postsynaptic membrane; protein complex

Molecular Function: ATP binding; enzyme binding; GTPase activator activity; kinase activity; protein binding; protein serine/threonine kinase activity; protein-tyrosine kinase activity; Rho guanyl-nucleotide exchange factor activity

Biological Process: actin cytoskeleton organization and biogenesis; brain development; inner ear morphogenesis; negative regulation of cellular extravasation; negative regulation of inflammatory response; negative regulation of neutrophil degranulation; neuromuscular process controlling balance; peptidyl-tyrosine phosphorylation; platelet-derived growth factor receptor signaling pathway; positive regulation of GTPase activity; positive regulation of phagocytosis; protein amino acid autophosphorylation; protein amino acid phosphorylation; regulation of cell cycle; regulation of Rho protein signal transduction; regulation of small GTPase mediated signal transduction; regulation of vascular permeability; response to lipopolysaccharide; signal transduction

Disease: Leukemia, Acute Lymphoblastic; Leukemia, Chronic Myeloid