HEMOGLOBIN DEBROUSSE (BETA-96[FG3]LEU-]PRO) - A NEW UNSTABLE HEMOGLOBIN WITH TWOFOLD INCREASED OXYGEN-AFFINITY

Authors
LACAN P KISTER J FRANCINA A SOUILLET G GALACTEROS F DELAUNAY J WAJCMAN H
Citation
P. Lacan et al., HEMOGLOBIN DEBROUSSE (BETA-96[FG3]LEU-]PRO) - A NEW UNSTABLE HEMOGLOBIN WITH TWOFOLD INCREASED OXYGEN-AFFINITY, American journal of hematology, 51(4), 1996, pp. 276-281
Citations number
15
Categorie Soggetti
Hematology
Journal title
American journal of hematology → ACNP
ISSN journal
03618609
Volume
51
Issue
4
Year of publication
1996
Pages
276 - 281
Database
ISI
SICI code
0361-8609(1996)51:4<276:HD(-AN>2.0.ZU;2-W
Abstract
Hemoglobin Debrousse (beta 96[FG3]Leu --> Pro) is a new unstable varia nt, with high oxygen affinity responsible, in the steady state, for an apparently well-compensated chronic hemolytic anemia, The functional properties of this variant are due to the replacement of a leucine res idue which is involved in the hydrophobic environment of the proximal side of the heme, This electrophoretically neutral hemoglobin was foun d as a de novo case in a g-year-old girl suffering from severe anemia with hemolysis and transient aplastic crisis, following infection by p arvovirus B19. (C) 1996 Wiley-Liss, Inc.