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

doi:10.1016/0167-4838(85)90043-3

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

Hematology and Oncology

Research output: Contribution to journal › Article

13 Scopus citations

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 language	English (US)
Pages (from-to)	257-260
Number of pages	4
Journal	Biochimica et Biophysica Acta (BBA)/Protein Structure and Molecular
Volume	831
Issue number	2
DOIs	https://doi.org/10.1016/0167-4838(85)90043-3
Publication status	Published - 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

Cite this

Kamel, K., El-Najjar, A., Webber, B. B., Chen, S. S., Wilson, J. B., Kutlar, A., & Huisman, T. H. J. (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, 831(2), 257-260. https://doi.org/10.1016/0167-4838(85)90043-3

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, A.; 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

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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 - Webber, B. B.

AU - Chen, S. S.

AU - Wilson, J. B.

AU - Kutlar, A.

AU - Huisman, T. H.J.

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10.1016/0167-4838(85)90043-3