Proceedings of the National Academy of Sciences · 1992 · 181 citations · 35 references
Adaptive Immune SystemMixed-phenotype Acute LeukemiaImmunologyPathologyCd4 T Cell ResponsesInnate ImmunityImmunotherapyT-cell ImmunityTumor BiologyMyeloid NeoplasiaHematological MalignancyHematologyCell SignalingBcr GeneAutoimmunityT Cell ImmunityCell BiologyMalignant Blood DisorderBcr GenesAdult T-cell Leukemia-lymphomaBcr Amino AcidsMedicineCellular Immune Response
Chronic myelogenous leukemia is driven by the t(9;22)(q34;q11) translocation that fuses the BCR and ABL genes into a 210‑kDa chimeric protein with abnormal tyrosine‑kinase activity, and the unique joining region of this protein contains sequences expressed only by malignant cells. The study shows that synthetic peptides from the BCR‑ABL joining region elicit CD4⁺, MHC‑II–restricted T cells that recognize only the combined BCR‑ABL sequence, proliferate in response to the full protein, and thus identify the protein as a tumor‑specific antigen.
The hallmark of chronic myelogenous leukemia is the translocation of the human c-abl protooncogene (ABL) from chromosome 9 to the specific breakpoint cluster region (bcr) of the BCR gene on chromosome 22. The t(9;22)(q34;q11) translocation results in the formation of a BCR-ABL fusion gene that encodes a 210-kDa chimeric protein with abnormal tyrosine kinase activity. The ABL and BCR genes are expressed by normal cells and thus the encoded proteins are presumably nonimmunogenic. However, the joining-region segment of the p210BCR-ABL chimeric protein is composed of unique sequences of ABL amino acids joined to BCR amino acids that are expressed only by malignant cells. The current study demonstrates that the joining region of BCR-ABL protein is immunogenic to murine T cells. Immunization of mice with synthetic peptides corresponding to the joining region elicited peptide-specific, CD4+, class II major histocompatibility complex-restricted T cells. The BCR-ABL peptide-specific T cells recognized only the combined sequence of BCR-ABL amino acids and not BCR or ABL amino acid sequences alone. Importantly, the BCR-ABL peptide-specific T cells could recognize and proliferate in response to p210BCR-ABL protein. The response of peptide-specific T cells to protein demonstrated that p210BCR-ABL can be processed by antigen-presenting cells so that the joining segment is bound to class II major histocompatibility complex molecules in a configuration similar to that of the immunizing peptide and in a concentration high enough to stimulate the antigen-specific T-cell receptor. Thus, BCR-ABL protein represents a potential tumor-specific antigen related to the transforming event and shared by many individuals with chronic myelogenous leukemia.
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Fused transcript of abl and bcr genes in chronic myelogenous leukaemia
Emma Shtivelman, Batia Lifshitz, Robert Peter Gale et al. · Nature · 1985 · 1.6K citations
Philip J. Fialkow, Robert J. Jacobson, Thalia Papayannopoulou · The American Journal of Medicine · 1977 · 876 citations
Acute leukaemia in bcr/abl transgenic mice
Nora Heisterkamp, Guido Jenster, Joanne ten Hoeve et al. · Nature · 1990 · 683 citations
Hematological Malignancy, Acute Leukaemia, Mixed-phenotype Acute Leukemia +3