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BIBTEX RIS

Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity

2012; Elsevier BV; Volume: 519; Issue: 1 Linguagem: Inglês

10.1016/j.abb.2012.01.001

ISSN

1096-0384

Autores

Susan E. Jorge, Ariel A. Petruk, Elza Kimura, Denise M. Oliveira, Lucas Caire da Silva, C.N. Suemasu, Paulo Augusto Achucarro Silveira, Dulcinéia Martins de Albuquerque, Fernando Ferreira Costa, Munir S. Skaf, Leandro Martı́nez, María de Fátima Sonati,

Tópico(s)

Iron Metabolism and Disorders

Resumo

Hb S-São Paulo (SP) [HBB:c.20A > T p.Glu6Val; c.196A > G p.Lys65Glu] is a new double-mutant hemoglobin that was found in heterozygosis in an 18-month-old Brazilian male with moderate anemia. It behaves like Hb S in acid electrophoresis, isoelectric focusing and solubility testing but shows different behavior in alkaline electrophoresis, cation-exchange HPLC and RP-HPLC. The variant is slightly unstable, showed reduced oxygen affinity and also appeared to form polymers more stable than the Hb S. Molecular dynamics simulation suggests that the polymerization is favored by interfacial electrostatic interactions. This provides a plausible explanation for some of the reported experimental observations.

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