Hemoglobin Hotel-Dieu beta 99 Asp replaced by Gly (g1). A new abnormal hemoglobin with high oxygen affinity

Hemoglobin. 1981;5(1):19-31. doi: 10.3109/03630268108996908.

Abstract

Hemoglobin Hotel-Dieu was detected by isoelectric focusing during investigation of a patient who had erythrocytosis. This variant migrates on cellulose acetate electrophoresis to a cathodic position relative to Hb F. In hemoglobin Hotel-Dieu, aspartic acid is substituted by glycine in position 99 of the beta chain. As in other abnormal hemoglobins in which substitution of this residue has occurred, Hb Hotel-Dieu exhibits a high oxygen affinity and is associated with familial erythrocytosis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Amino Acids / analysis
  • Female
  • Genetic Variation
  • Hemoglobins / analysis
  • Hemoglobins, Abnormal / isolation & purification
  • Hemoglobins, Abnormal / metabolism*
  • Humans
  • Male
  • Oxygen / blood
  • Pedigree
  • Peptide Fragments
  • Polycythemia / blood*

Substances

  • Amino Acids
  • Hemoglobins
  • Hemoglobins, Abnormal
  • Peptide Fragments
  • hemoglobin Hotel-Dieu
  • Oxygen