Pharmacologic induction of fetal hemoglobin synthesis

Cellular and molecular mechanisms

Yih Ming Yang, Betty Sue Pace

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

The switch from embryonic to fetal then to adult hemoglobin synthesis is a unique phenomenon during early human development. Fetal hemoglobin (Hb F) is known to interfere with polymerization of Hb S in erythrocytes. Several pharmacologic agents such as 5-azacytidine, myleran, hydroxyurea, erthropoietin, and butyrates enhance fetal hemoglobin production and have been used in hemoglobinopathy patients to ameliorate severe pain episodes and reduce severe anemia. Among these, hydroxyurea is the agent of choice because of its safety and ease of administration. One of the primary cellular mechanisms involved in pharmacologic induction of Hb F synthesis is rapid regeneration of erythroid precursors following the cytoreduction phase of certain pharmacologic agents. Molecular mechanisms involving changes in chromatin structure and/or transcription factor binding have been demonstrated for γ gene induction by butyrate. Identifying the proteins involved in γ gene activation by various compounds may offer a new strategy for gene therapy to cure hemoglobinopathy disorders.

Original languageEnglish (US)
Pages (from-to)87-106
Number of pages20
JournalFetal and Pediatric Pathology
Volume20
Issue number1
DOIs
StatePublished - Dec 1 2001
Externally publishedYes

Fingerprint

Fetal Hemoglobin
Hemoglobinopathies
Hydroxyurea
Butyrates
Azacitidine
Busulfan
Human Development
Polymerization
Genetic Therapy
Transcriptional Activation
Chromatin
Anemia
Regeneration
Hemoglobins
Transcription Factors
Erythrocytes
Safety
Pain
Genes
Proteins

Keywords

  • γ globin
  • fetal hemoglobin
  • hemoglobinopathy
  • induction
  • pharmacologic

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

Cite this

Pharmacologic induction of fetal hemoglobin synthesis : Cellular and molecular mechanisms. / Yang, Yih Ming; Pace, Betty Sue.

In: Fetal and Pediatric Pathology, Vol. 20, No. 1, 01.12.2001, p. 87-106.

Research output: Contribution to journalArticle

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