Phosphorylation of BCR moiety of BCR-FGFR1

Stable Identifier
R-HSA-1839067
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
Summation

Unique among FGFR1 fusion proteins, which generally give rise to an atypical myeloproliferative syndrome (EMS) (reviewed in Jackson, 2010), the BCR-FGFR1 fusion results in a more typical chronic myeloid leukemia (CML). Although both EMS and CML activate PLCgamma signaling, and mutation of the FGFR1 Y766 PLCgamma binding site attenuates both diseases, CML-specific signaling also appears to be mediated through the BCR portion of the fusion protein. BCR Y177 binds GRB2-GAB1 and induces CML-like leukemia in mice, while expression of a Y177F BCR-FGFR1 fusion induces EMS-like disease (Roumiantsev, 2004).

Literature References
PubMed ID Title Journal Year
15050920 Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations

Roumiantsev, S, Krause, DS, Neumann, CA, Dimitri, CA, Asiedu, F, Cross, NC, Van Etten, RA

Cancer Cell 2004
20226962 8p11 myeloproliferative syndrome: a review

Jackson, CC, Medeiros, LJ, Miranda, RN

Hum Pathol 2010
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
protein tyrosine kinase activity of BCR-p-FGFR1 fusion mutant dimer [cytosol]
Physical Entity
Activity
Disease
Name Identifier Synonyms
chronic myeloid leukemia 8552 CML, chronic myelogenous leukemia, no ICD-O subtype (morphologic abnormality), chronic myeloid leukemia, disease (disorder), chronic myeloid leukemia without mention of remission, CML - chronic Myelogenous Leukemia, chronic myeloid leukemia in remission (disorder), Myeloid Leukemia, chronic, CML in Remission, chronic myeloid leukemia in remission, chronic myeloid leukaemia, chronic myeloid leukemia NOS (disorder)
cancer 162 malignant tumor, malignant neoplasm, primary cancer
Authored
Reviewed