Literature DB >> 857849

Haemoglobin Radcliffe (alpha2beta299(Gi)Ala): a high oxygen-affinity variant causing familial polycythaemia.

D J Weatherall, J B Clegg, S T Callender, R M Wells, R E Gale, E R Huehns, M F Perutz, G Viggiano, C Ho.   

Abstract

Three members of an Oxfordshire family have polycythaemia. In each case their whole-blood oxygen affinity is increased. This is due to a previously undescribed haemoglobin variant which has been named haemoglobin Radcliffe (alpha2beta299(Gl)Ala). In addition to having a high oxygen affinity haemoglobin Radcliffe shows virtually no haem-haem interaction and a diminished Bohr effect. It is synthesized at the same rate and is as stable as haemoglobin A. X-ray analysis indicates that crystals of deoxyhaemoglobin Radcliffe are isomorphous with those of deoxyhaemoglobin A. Solutions of haemoglobin Radcliffe were also studied by high-resolution proton nuclear magnetic resonance spectroscopy. The structure/function relationships of haemoglobin Radcliffe are discussed in the light of these studies.

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Year:  1977        PMID: 857849     DOI: 10.1111/j.1365-2141.1977.tb00575.x

Source DB:  PubMed          Journal:  Br J Haematol        ISSN: 0007-1048            Impact factor:   6.998


  4 in total

1.  High affinity haemoglobins.

Authors: 
Journal:  Br Med J       Date:  1977-10-22

2.  Restoring allosterism with compensatory mutations in hemoglobin.

Authors:  H W Kim; T J Shen; D P Sun; N T Ho; M Madrid; M F Tam; M Zou; P F Cottam; C Ho
Journal:  Proc Natl Acad Sci U S A       Date:  1994-11-22       Impact factor: 11.205

3.  Diagnosis and management of congenital and idiopathic erythrocytosis.

Authors:  Mary Frances McMullin
Journal:  Ther Adv Hematol       Date:  2012-12

4.  Properties of a recombinant human hemoglobin with aspartic acid 99(beta), an important intersubunit contact site, substituted by lysine.

Authors:  H Yanase; S Cahill; J J Martin de Llano; L R Manning; K Schneider; B T Chait; K D Vandegriff; R M Winslow; J M Manning
Journal:  Protein Sci       Date:  1994-08       Impact factor: 6.725

  4 in total

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