title: Structure-function of Hb Marseille-Long Island [alpha 2 beta 2 N-methionyl-2(NA2) His----Pro].
abstract: Suspensions of red cells containing Hb Marseille-Long Island showed decreased oxygen affinity and low interaction with 2,3-diphosphoglycerate. Oxygen equilibrium studies of the purified component confirmed these abnormalities. Oxidation rate measurements of carbonmonoxy-Hb Marseille and carbonmonoxy-Hb A by ferricyanide showed an increased rate for the former, suggesting an increased dissociation constant for carbon monoxide. Nuclear Magnetic Resonance spectra in the high field region revealed small changes in the proximal region of the heme pocket. These results indicated that the mutation causes a perturbation at a distance from the mutation site.
authors.affiliation: INSERM U.299, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: B
authors.initials: B
authors.lastname: Bohn
authors.forename: G
authors.initials: G
authors.lastname: Craescu
authors.forename: J
authors.initials: J
authors.lastname: Kister
authors.forename: J
authors.initials: J
authors.lastname: Bardakdjian
authors.forename: Y
authors.initials: Y
authors.lastname: Blouquit
authors.forename: J
authors.initials: J
authors.lastname: Rosa
authors.forename: C
authors.initials: C
authors.lastname: Poyart
authors.forename: F
authors.initials: F
authors.lastname: Galacteros
mesh_terms: D019794:2,3-Diphosphoglycerate; D000328:Adult; D004163:Diphosphoglyceric Acids / Q000097:blood*; D005260:Female; D006441:Hemoglobin A / Q000378:metabolism; D006455:Hemoglobins, Abnormal / Q000378:metabolism*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007700:Kinetics; D046911:Macromolecular Substances; D009682:Magnetic Resonance Spectroscopy; D010100:Oxygen / Q000097:blood*; D013329:Structure-Activity Relationship
chemical_list: D004163:Diphosphoglyceric Acids; D006455:Hemoglobins, Abnormal; D046911:Macromolecular Substances; D019794:2,3-Diphosphoglycerate; D006441:Hemoglobin A; C044001:hemoglobin Marseille; D010100:Oxygen