title: [Advances on pathogenesis research of acute myeloid leukemia with t(8;21)-- review].
abstract: Acquired chromosomal translocations can be identified in nearly 50% of human acute myeloid leukemias. The common chromosomal translocation in this disease is t(8;21) (q22;q22). It involves the aml1 (runx1) gene on chromosome 21 and the eto (mtg8, runx1t1) gene on chromosome 8 generating the aml1/eto fusion gene. An initial model for its pathogenesis emphasized the conversion of a hematopoietic transcriptional activator AML1 into a leukemogenic repressor which blocked myeloid differentiation at the level of target gene regulation. Aml1/eto fusion genes inhibit key hematopoietic transcription factor that function as tumor suppressors at several nodal point during hematopoietic differentiation. A new model is presented in which aml1/eto coordinates expansion of the stem cell compartment with diminished lineage commitment and with genome instability. In this review, the molecular role of aml1/eto fusion gene and his transcribed isoforms in regulating stem renewal, blocking hematopoietic differentiation and interacting with various lineage-specific transcription factors are summarized.
authors.affiliation: Chinese Academy of Medical Sciences, Tianjin 30020, China.
authors.forename: Hui-Min
authors.initials: HM
authors.lastname: Zeng
authors.forename: Ye
authors.initials: Y
authors.lastname: Guo
authors.forename: Xiao-Fan
authors.initials: XF
authors.lastname: Zhu
mesh_terms: D002891:Chromosomes, Human, Pair 21; D002898:Chromosomes, Human, Pair 8; D050676:Core Binding Factor Alpha 2 Subunit / Q000235:genetics*; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000235:genetics* / Q000473:pathology*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D000075142:RUNX1 Translocation Partner 1 Protein
chemical_list: C107844:AML1-ETO fusion protein, human; D050676:Core Binding Factor Alpha 2 Subunit; D015514:Oncogene Proteins, Fusion; D000075142:RUNX1 Translocation Partner 1 Protein