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      Hemoglobin Rouen (alpha-140 (HC2) Tyr-->His): alteration of the alpha-chain C-terminal region and moderate increase in oxygen affinity.

      Biochimica et Biophysica Acta
      Aged, Aged, 80 and over, Allosteric Regulation, Amino Acids, analysis, Carbon Monoxide, metabolism, Chromatography, High Pressure Liquid, Hemoglobins, Abnormal, chemistry, genetics, Histidine, Humans, Kinetics, Male, Mutation, Oxygen, Polycythemia Vera, blood, Serine Endopeptidases, Tyrosine

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          Abstract

          Hb Rouen (alpha 140(HC2) Tyr-->His) is a moderately high oxygen-affinity variant that was found in coincidence with polycythemia vera in a French patient. This hemoglobin provides an example of an alteration of the C-terminus of the alpha-chain, a region involved in the mechanisms of allosteric regulation. The increase in oxygen-affinity and decrease in cooperativity of this variant is much smaller than that resulting from the same substitution in the beta-chain. This model provides additional evidence for the inequivalence between the alpha- and beta-subunits.

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