RUNX1 and RUNX1-ETO: roles in hematopoiesis and leukemogenesis.
RUNX1 is a transcription factor that regulates critical processes in many aspects of hematopoiesis. RUNX1 is also integral in defining the definitive hematopoietic stem cell. In addition, many hematological diseases like myelodysplastic syndrome and myeloproliferative neoplasms have been associated with mutations in RUNX1. Located on chromosomal 21, the RUNX1 gene is involved in many forms of chromosomal translocations in leukemia. t(8;21) is one of the most common chromosomal translocations found in acute myeloid leukemia (AML), where it results in a fusion protein between RUNX1 and ETO. The RUNX1-ETO fusion protein is found in approximately 12% of all AML patients. In this review, we detail the structural features, functions, and models used to study both RUNX1 and RUNX1-ETO in hematopoiesis over the past two decades.
Moores Cancer Center, Department of Pathology and Division of Biological Sciences, University of California, San Diego, La Jolla, CA 92093, USA.
Kentson
K
Lam
Dong-Er
DE
Zhang
D050676:Core Binding Factor Alpha 2 Subunit / Q000737:chemistry / Q000502:physiology*; D006410:Hematopoiesis / Q000502:physiology*; D006801:Humans; D007938:Leukemia / Q000209:etiology* / Q000503:physiopathology; D009369:Neoplasms / Q000235:genetics; D011487:Protein Conformation; D011518:Proto-Oncogene Proteins / Q000502:physiology*; D000075142:RUNX1 Translocation Partner 1 Protein; D014157:Transcription Factors / Q000502:physiology*; D014178:Translocation, Genetic
D050676:Core Binding Factor Alpha 2 Subunit; D011518:Proto-Oncogene Proteins; D000075142:RUNX1 Translocation Partner 1 Protein; C493728:RUNX1 protein, human; C082222:RUNX1T1 protein, human; D014157:Transcription Factors