Sanna, Maria Teresa and Giardina, Bruno and Scatena, Roberto and Pellegrini, Mariagiuseppina and Olianas, Alessandra and Manca, Laura and Masala, Bruno Lucio and Castagnola, Massimo and Corda, Marcella (1994) Functional alterations in adult and fetal hemoglobin Sassari Asp-α126(H9)→His: the role of α1 α2 contact. The Journal of Biological Chemistry, Vol. 269 (28), p. 18338-18342. eISSN 1083-351X. Article.
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The effects of pH, organic phosphates (2,3-diphosphoglycerate), and temperature on the functional properties of both adult and fetal hemoglobin Sassari α (Asp-126→His) have been studied. The functional properties of the adult variant are characterized by the following: (i) an oxygen affinity higher than that of normal HbA in all the experimental conditions used; (ii) a dramatic reduction of homotropic interactions (n50 very close to unity); and (iii) a significant decrease of the effect of 2,3-diphosphoglycerate, which is 35% lower than that observed on HbA. The fetal variant shows an increased oxygen affinity compared with normal HbF and an almost abolished heme-heme interaction. The molecular basis of these functional differences is discussed in terms of the possible role played by the substitution of α (Asp-26→His) on the stability of the R state of the molecule due to a decreased interaction at the level of α1 α2 contact.
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