breakpoint cluster region protein P160 (p160BCR)
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Introduction
Protein product of BCR-gene (breakpoint cluster region) gene that forms a chimeric gene with abl in the Philadelphia chromosome associated with chronic myelogenous leukemia (CML).
Function
- GTPase-activating protein for RAC1 & CDC42
- promotes exchange of RAC or CDC42-bound GDP by GTP, thereby activating them
- displays serine/threonine kinase activity
- interacts with PDZK1
- may interact with CCPG1
- autophosphorylated
ATP + a protein <--> ADP + a phosphoprotein
Structure
- homotetramer
- region involved in binding to ABL1 SH2-domain is rich in Ser & needs to be Ser/Thr phosphorylated prior to SH2 binding; this region is essential for the activation of the ABL1 tyrosine kinase & transforming potential of the chimeric BCR-ABL oncogene
- DH domain is involved in interaction with CCPG1
- contains 1 C2 domain contains 1 DH (DBL-homology) domain contains 1 PH domain contains 1 Rho-GAP domain
Alternative splicing
named isoforms=2
Pathology
- chromosomal translocation t(9;22)(q34.1;q11) involving BCR with ABL1 is a cause of chronic myeloid leukemia translocation produces a BCR-ABL found also in acute myeloid leukemia (AML) & acute lymphoblastic leukemia (ALL))
More general terms
More specific terms
Additional terms
References
- ↑ Maru Y, Witte ON. The BCR gene encodes a novel serine/threonine kinase activity within a single exon. Cell. 1991 Nov 1;67(3):459-68. PMID: https://www.ncbi.nlm.nih.gov/pubmed/1657398
- ↑ Musacchio A, Gibson T, Rice P, Thompson J, Saraste M. The PH domain: a common piece in the structural patchwork of signalling proteins. Trends Biochem Sci. 1993 Sep;18(9):343-8. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/8236453
- ↑ Atlas of Genetics & cytogenetics in oncology & Haematology http://atlasgeneticsoncology.org/Genes/BCR.html