Molecular Classification
Tyrosine kinase, Fusion protein, Oncoprotein
Other Names
Philadelphia chromosome, p210 BCR-ABL, p185 BCR-ABL, p230 BCR-ABL
Disease Roles
BCR-ABL fusion protein Overview
The BCR-ABL fusion protein is an abnormal, constitutively active tyrosine kinase resulting from a chromosomal translocation (the Philadelphia chromosome). This fusion leads to uncontrolled cell proliferation and is a hallmark of chronic myeloid leukemia (CML) and other leukemias. BCR-ABL is a major therapeutic target, with tyrosine kinase inhibitors (TKIs) designed to specifically inhibit its activity.
Mechanism of Action
Tyrosine kinase inhibitors (TKIs) bind to the ATP-binding site of the BCR-ABL kinase, preventing its activity and downstream signaling.
Biological Functions
Cell proliferation
Inhibition of apoptosis
Signal transduction
Leukemogenesis
Tyrosine kinase activity
Disease Associations
Chronic myeloid leukemia (CML)
Acute lymphoblastic leukemia (ALL)
Mixed phenotype acute leukemia (MPAL)
Acute myeloid leukemia (AML)
Safety Considerations
- TKI resistance (due to mutations in the ABL kinase domain)
- Drug-drug interactions
- Cardiovascular side effects (e.g., QT prolongation)
- Myelosuppression
- Pleural effusion
- Edema
Interacting Drugs
Imatinib
Dasatinib
Nilotinib
Bosutinib
Ponatinib
Asciminib
Associated Biomarkers
Biomarker |
---|
BCR-ABL transcript levels (qPCR) |
BCR-ABL protein expression |
Philadelphia chromosome detection (cytogenetics/FISH) |
ABL kinase domain mutations (for TKI resistance) |