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, Abdullah Kutlar, T. H.J. Huisman

Research output: Contribution to journal › Article

13 Citations (Scopus)

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
StatePublished - Oct 4 1985

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Abnormal Hemoglobins
Heterozygote
Structural analysis
Methionine
Glutamic Acid
Hemoglobins
Substitution reactions
Molecules
hemoglobin Doha

Keywords

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

ASJC Scopus subject areas

  • Structural Biology
  • Biophysics
  • Biochemistry
  • Molecular Biology

Cite this

Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female. / Kamel, K.; El-Najjar, A.; Webber, B. B.; Chen, S. S.; Wilson, J. B.; Kutlar, Abdullah; Huisman, T. H.J.

In: Biochimica et Biophysica Acta (BBA)/Protein Structure and Molecular, Vol. 831, No. 2, 04.10.1985, p. 257-260.

Research output: Contribution to journal › Article

Kamel, K, El-Najjar, A, Webber, BB, Chen, SS, Wilson, JB, Kutlar, A & Huisman, THJ 1985, 'Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female', Biochimica et Biophysica Acta (BBA)/Protein Structure and Molecular, vol. 831, no. 2, pp. 257-260. https://doi.org/10.1016/0167-4838(85)90043-3
Kamel K, El-Najjar A, Webber BB, Chen SS, Wilson JB, Kutlar A et al. Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female. Biochimica et Biophysica Acta (BBA)/Protein Structure and Molecular. 1985 Oct 4;831(2):257-260. https://doi.org/10.1016/0167-4838(85)90043-3
Kamel, K. ; El-Najjar, A. ; Webber, B. B. ; Chen, S. S. ; Wilson, J. B. ; Kutlar, Abdullah ; Huisman, T. H.J. / Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female. In: Biochimica et Biophysica Acta (BBA)/Protein Structure and Molecular. 1985 ; Vol. 831, No. 2. pp. 257-260.
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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.",
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AU - Kamel, K.

AU - El-Najjar, A.

AU - Webber, B. B.

AU - Chen, S. S.

AU - Wilson, J. B.

AU - Kutlar, Abdullah

AU - Huisman, T. H.J.

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AB - 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.

KW - (Human)

KW - Amino acid sequence

KW - Blocking group

KW - Hemoglobin variant

KW - Methionine

KW - N-terminal

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