title: Mouse alpha chains inhibit polymerization of hemoglobin induced by human beta S or beta S Antilles chains.
abstract: A murine model of sickle cell disease was tested by studying the polymerization of hybrid hemoglobin tetramers between alpha mouse and human beta S or beta S Antilles chains were prepared from Hb S Antilles, which was a new sickling hemoglobin inducing a sickle cell syndrome more severe than Hb S. The hybrid molecules did not polymerize in solution, indicating that the mouse alpha chains inhibited fiber formation. Consequently, a mouse model for sickle cell disease requires the transfer and expression of both alpha and beta S or beta S Antilles genes.
authors.affiliation: INSERM U.91, CNRS EA 607, Hôpital Henri Mondor, Créteil, France.
authors.forename: M D
authors.initials: MD
authors.lastname: Rhoda
authors.forename: C
authors.initials: C
authors.lastname: Domenget
authors.forename: M
authors.initials: M
authors.lastname: Vidaud
authors.forename: J
authors.initials: J
authors.lastname: Bardakdjian-Michau
authors.forename: P
authors.initials: P
authors.lastname: Rouyer-Fessard
authors.forename: J
authors.initials: J
authors.lastname: Rosa
authors.forename: Y
authors.initials: Y
authors.lastname: Beuzard
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D006451:Hemoglobin, Sickle / Q000302:isolation & purification / Q000378:metabolism*; D006801:Humans; D046911:Macromolecular Substances; D051379:Mice; D010108:Oxyhemoglobins / Q000378:metabolism; D055503:Protein Multimerization; D013045:Species Specificity
chemical_list: D006451:Hemoglobin, Sickle; D046911:Macromolecular Substances; D010108:Oxyhemoglobins; C055657:hemoglobin S-Antilles