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Improvement of mouse β thalassaemia by hydroxyurea
Author(s) -
Sauvage Claire,
RouyerFessard Philippe,
Beuzard Yves
Publication year - 1993
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1993.tb03106.x
Subject(s) - ankyrin , reticulocyte , spectrin , hydroxycarbamide , heinz body , red blood cell , beta thalassaemia , medicine , phenotype , hemoglobin , endocrinology , andrology , immunology , biology , thalassemia , cell , biochemistry , chemotherapy , gene , cytoskeleton , messenger rna
Summary. The present report provides evidence that hydroxyurea (HU) improves the β 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 β minor/α 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 α chains decreased, the proportion of spectrin and ankyrin increased and red cell deformability also increased.

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