Abstract
RUNX1/AML1, located on chromosome 21, is a key factor in the generation and maintenance of hematopoietic stem cells and the gene most frequently implicated in human leukemias. Chromosome translocations and point mutations are well-documented genetic alterations in RUNX leukemia (also known as CBF leukemia). In addition, overdosage or overexpression of RUNX1 is suspected to be a third mode of RUNX1 involvement in leukemogenesis. The possibility that this mode might underlie Down syndrome-related leukemias caused by trisomy of chromosome 21 is discussed.
Publication types
-
Research Support, Non-U.S. Gov't
-
Review
MeSH terms
-
Animals
-
B-Lymphocytes / metabolism
-
Basic-Leucine Zipper Transcription Factors / metabolism
-
Core Binding Factor Alpha 1 Subunit / genetics
-
Core Binding Factor Alpha 1 Subunit / metabolism
-
Core Binding Factor Alpha 2 Subunit / genetics
-
Core Binding Factor Alpha 2 Subunit / metabolism*
-
DNA-Binding Proteins / metabolism
-
Down Syndrome / complications
-
Down Syndrome / genetics*
-
Fanconi Anemia Complementation Group Proteins / metabolism
-
GATA1 Transcription Factor / genetics
-
Gene Dosage*
-
Gene Expression Regulation, Leukemic
-
Humans
-
Leukemia / genetics*
-
Leukemia, Megakaryoblastic, Acute / genetics
-
Lymphoma / genetics
-
Mice
-
Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
-
Proto-Oncogene Protein c-ets-2 / metabolism
-
Retroviridae / genetics
-
Thrombopoiesis / genetics
-
Trans-Activators / metabolism
-
Transcriptional Regulator ERG
Substances
-
BACH1 protein, human
-
Basic-Leucine Zipper Transcription Factors
-
Core Binding Factor Alpha 1 Subunit
-
Core Binding Factor Alpha 2 Subunit
-
DNA-Binding Proteins
-
ERG protein, human
-
ETS2 protein, human
-
Fanconi Anemia Complementation Group Proteins
-
GATA1 Transcription Factor
-
Proto-Oncogene Protein c-ets-2
-
RUNX1 protein, human
-
Trans-Activators
-
Transcriptional Regulator ERG