| Literature DB >> 22244832 |
Susan E D C Jorge1, Ariel A Petruk, Elza M Kimura, Denise M Oliveira, Lucas Caire, Cintia N Suemasu, Paulo A A Silveira, Dulcineia M Albuquerque, Fernando F Costa, Munir S Skaf, Leandro Martínez, Maria de Fatima Sonati.
Abstract
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.Entities:
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Year: 2012 PMID： 22244832 DOI： 10.1016/j.abb.2012.01.001
Source DB: PubMed Journal: Arch Biochem Biophys ISSN： 0003-9861 Impact factor: 4.013