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 journalArticle

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

Fingerprint

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 journalArticle

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.
@article{1c2e2947f2da4bb7b75560d1b4ebe745,
title = "Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female",
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.",
keywords = "(Human), Amino acid sequence, Blocking group, Hemoglobin variant, Methionine, N-terminal",
author = "K. Kamel and A. El-Najjar and Webber, {B. B.} and Chen, {S. S.} and Wilson, {J. B.} and Abdullah Kutlar and Huisman, {T. H.J.}",
year = "1985",
month = "10",
day = "4",
doi = "10.1016/0167-4838(85)90043-3",
language = "English (US)",
volume = "831",
pages = "257--260",
journal = "Biochimica et Biophysica Acta - Proteins and Proteomics",
issn = "1570-9639",
publisher = "Elsevier",
number = "2",

}

TY - JOUR

T1 - Hb Doha or α2β2[X-N-Met-1-(NA1)Val → Glu]; a new β-chain abnormal hemoglobin observed in a Qatari female

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.

PY - 1985/10/4

Y1 - 1985/10/4

N2 - 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.

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

UR - http://www.scopus.com/inward/record.url?scp=0021931068&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0021931068&partnerID=8YFLogxK

U2 - 10.1016/0167-4838(85)90043-3

DO - 10.1016/0167-4838(85)90043-3

M3 - Article

C2 - 3840039

AN - SCOPUS:0021931068

VL - 831

SP - 257

EP - 260

JO - Biochimica et Biophysica Acta - Proteins and Proteomics

JF - Biochimica et Biophysica Acta - Proteins and Proteomics

SN - 1570-9639

IS - 2

ER -