Hemoglobin debrousse (β96[FG3]Leu → Pro): A new unstable hemoglobin with twofold increased oxygen affinity
✍ Scribed by Lacan, Philippe; Kister, Jean; Francina, Alain; Souillet, Gérard; Galactéros, Frédéric; Delaunay, Jean; Wajcman, Henri
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 484 KB
- Volume
- 51
- Category
- Article
- ISSN
- 0361-8609
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✦ Synopsis
Hemoglobin Debrousse (p96[FG3]Leu + Pro) is a new unstable variant, with high oxygen affinity responsible, In the steady state, for an apparently weii-compensated chronic hemolytic anemia. The functional properties of this variant are due to the replacement of a leucine residue which is Involved in the hydrophobic environment of the proximal side of the heme. This eiectrophoreticaily neutral hemoglobin was found as a de novo case in a Gyear-old girl suffering from severe anemia with hemolysis and transient aplastic crisis, following infection by parvovirus B19.