Hemoglobin bart's disease in an italian boy: Interaction between α-thalassemia and hereditary persistence of fetal hemoglobin

David H.k. Chui, Margaret Patterson, Carol E. Dowling, Haig H. Kazazian, Alan G. Kendall

Research output: Contribution to journalArticle


HEMOGLOBIN is a tetramer consisting of two pairs of globin chains, each of which is associated with a heme group. In adults, the predominant hemoglobin — hemoglobin A — is composed of two α-globin and two β-globin chains (α2β2); the principal fetal hemoglobin — hemoglobin F — is made up of two α-globin and two γ-globin chains (α2γ2). α-Globin chains are coded for by two adjacent α-globin genes on the short arm of each chromosome 16. The embryonic ζ-globin chains, which resemble α-globin chains, are coded for by a ζ-globin gene on.

Original languageEnglish (US)
Pages (from-to)179-182
Number of pages4
JournalNew England Journal of Medicine
Issue number3
StatePublished - Jul 19 1990

ASJC Scopus subject areas

  • Medicine(all)

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