Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female

K. Kamel, A. El-Najjar, B. B. Webber, S. S. Chen, J. B. Wilson, A. Kutlar, T. H.J. Huisman

Research output: Contribution to journalArticle

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Abstract

Structural analysis of a fast-moving hemoglobin variant, present in three members of a Qatari family, identified a Val → Glu substitution at position 1 (NA1) of the β-chain. The introduction of this glutamic acid residue prevents the removal of the initiator methione, thus extending the N-terminus by one residue to Met-Glu-His-Leu-Thr-. THe methionine residue is blocked by an as yet not completely identified molecule. The presence of the variant in a heterozygote does not have clinical consequences.

Original languageEnglish (US)
Pages (from-to)257-260
Number of pages4
JournalBiochimica et Biophysica Acta (BBA)/Protein Structure and Molecular
Volume831
Issue number2
DOIs
Publication statusPublished - Oct 4 1985

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Keywords

  • (Human)
  • Amino acid sequence
  • Blocking group
  • Hemoglobin variant
  • Methionine
  • N-terminal

ASJC Scopus subject areas

  • Biophysics
  • Structural Biology
  • Biochemistry
  • Molecular Biology

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