Transgenic mouse models of sickle cell disease.
An array of sickle cell syndromes has been obtained in transgenic mice, expressing HbS or super HbS, from the asymptomatic phenotype similar to the human A/S state to a syndrome more severe than the human homozygous S/S state, inducing 100% fetal death. Anemia was observed in SAD and SAD (beta th/ beta +) neonates and disappeared during postnatal development. In adults, many features of sickle cell disease are found in transgenic mice, especially in SAD and SAD (beta th/ beta +) mice, including abnormal hemolysis, vasoocclusion, microthrombosis, infarct, priapism, chronic organ defects, and death on hypoxia. These mouse models are relevant to the study of the pathophysiology of sickle cell disease and the induction of vasoocclusion and to evaluate new therapeutic approaches in vivo. Clotrimazole and Mg2+ restore hydration of sickle cells and 12 C79 protected SAD mice from lethal acute hypoxia.
Beuzard
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Institut d'Hématologie, Centre Hayem, Hôpital Saint-Louis, Paris, France.
D000755:Anemia, Sickle Cell / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D000818:Animals; D004195:Disease Models, Animal; D006451:Hemoglobin, Sickle / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics; D017086:beta-Thalassemia / Q000235:genetics* / Q000473:pathology / Q000628:therapy
D006451:Hemoglobin, Sickle; C070168:hemoglobin SAD; C055657:hemoglobin S-Antilles