Mammalian runt-domain proteins and their roles in hematopoiesis, osteogenesis, and leukemia

Jennifer J Westendorf, Scott W. Hiebert

Research output: Contribution to journalArticle

119 Citations (Scopus)

Abstract

Mammalian Runt-domain-containing factors are structurally and functionally similar and have essential roles in hematopoiesis and osteogenesis. These factors can act as either positive or negative transcriptional regulators of tissue-specific genes whose promoters or enhancers contain the consensus Runt-domain binding element, TGT/CGGT. This sequence is necessary but not sufficient to regulate the transcription of a wide variety of genes. Runt-domain factors are promoter organizers that cooperate with neighboring factors and recruit transcriptional co-activators or co-repressors to regulate expression of tissue-specific genes. AML1 is required for hematopoiesis and is a frequent target of chromosomal translocations in acute leukemias. Fusion proteins generated by these translocations are dominant repressors of genes regulated by the Runt-domain factors. AML3 may also be involved in leukemogenesis. In addition, AML3 has an essential role in bone development, as it is required for osteoblast differentiation and is mutated in patients with cleidocranial dysplasia.

Original languageEnglish (US)
Pages (from-to)51-58
Number of pages8
JournalJournal of Cellular Biochemistry
Volume76
Issue numberSUPPL. 32/33
StatePublished - 1999
Externally publishedYes

Fingerprint

Core Binding Factor alpha Subunits
Hematopoiesis
Osteogenesis
Leukemia
Genes
Cleidocranial Dysplasia
Dominant Genes
Co-Repressor Proteins
Genetic Translocation
Proteins
Bone Development
Protein Transport
Tissue
Osteoblasts
Transcription
Bone
Fusion reactions
Protein Domains

Keywords

  • AML1
  • AML2
  • AML3
  • Bone
  • CBFA
  • Cleidocranial dysplasia
  • ETO
  • Leukemia
  • Osteoblasts
  • PEPB2α
  • Runx

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology

Cite this

Mammalian runt-domain proteins and their roles in hematopoiesis, osteogenesis, and leukemia. / Westendorf, Jennifer J; Hiebert, Scott W.

In: Journal of Cellular Biochemistry, Vol. 76, No. SUPPL. 32/33, 1999, p. 51-58.

Research output: Contribution to journalArticle

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