title: Improvement of mouse beta thalassaemia by hydroxyurea.
abstract: The present report provides evidence that hydroxyurea (HU) improves the beta thalassaemic phenotype in mice receiving 200 mg/kg/d for 30 d. The haematocrit rose from 29 +/- 3% at day 0 to 37 +/- 4% at day 30 (P < 0.05), despite myelosuppression and decreased reticulocyte counts. The beta minor/alpha ratio of globin chain synthesis increased from 0.78 at day 0 to 0.97 at day 30 (P < 0.001). Membrane defects improved: the proportion of bound alpha chains decreased, the proportion of spectrin and ankyrin increased and red cell deformability also increased.
authors.affiliation: INSERM U.91, Hôpital Henri Mondor, Créteil, France.
authors.forename: C
authors.initials: C
authors.lastname: Sauvage
authors.forename: P
authors.initials: P
authors.lastname: Rouyer-Fessard
authors.forename: Y
authors.initials: Y
authors.lastname: Beuzard
mesh_terms: D000818:Animals; D001798:Blood Proteins / Q000187:drug effects; D004907:Erythrocyte Deformability / Q000187:drug effects; D004909:Erythrocyte Indices / Q000187:drug effects; D005914:Globins / Q000096:biosynthesis; D006400:Hematocrit; D006918:Hydroxyurea / Q000627:therapeutic use*; D007958:Leukocyte Count / Q000187:drug effects; D008565:Membrane Proteins / Q000187:drug effects; D051379:Mice; D008811:Mice, Inbred DBA; D017086:beta-Thalassemia / Q000097:blood / Q000188:drug therapy*
chemical_list: D001798:Blood Proteins; D008565:Membrane Proteins; D005914:Globins; D006918:Hydroxyurea