Wajcman H, Kister J, Marden M, Lahary A, Monconduit M, Galacteros F
INSERM U299, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
Biochim Biophys Acta. 1992 Oct 13;1180(1):53-7. doi: 10.1016/0925-4439(92)90026-j.
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.
