Studies on the oxygen transport in the clinical association between Hb-S and Hb-C

Panminerva Med. 1997 Sep;39(3):169-73.

Abstract

The whole blood oxygen affinity of a Negro carrier of SC disease was found to be characterized by some right-shifted p50 and clearly increased Bohr effect, whereas the isolated and purified Hb-S and Hb-C exhibited slight deficiencies mainly of the Bohr effect. The right-shifted p50 from whole blood can be easily explained by the mild anemia with a parallel increase of 2,3-diphosphoglycerate (DPG), whereas the functional discrepancies between whole blood function and that of the purified Hb-S and C could be due, at least in part, to the presence in vivo of consistent amounts of hybrid Hb tetramers of the type alpha alpha beta S beta C. Unfortunately, the mechanism promoting the formation (or dissolution) of hybrids are fundamentally unknown; so, either their presence and functional properties are very difficult to be explored.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Hemoglobin C / metabolism*
  • Hemoglobin SC Disease / blood
  • Hemoglobin, Sickle / metabolism*
  • Humans
  • Oxygen / blood*

Substances

  • Hemoglobin, Sickle
  • Hemoglobin C
  • Oxygen