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Type: Artigo de periódico
Title: Hb S-Sao Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
Author: Jorge, Susan E. D. C.
Petruk, Ariel A.
Kimura, Elza M.
Oliveira, Denise M.
Caire, Lucas
Suemasu, Cintia N.
Silveira, Paulo A. A.
Albuquerque, Dulcineia M.
Costa, Fernando F.
Skaf, Munir S.
Martinez, Leandro
Sonati, Maria de Fatima
Abstract: Hb S-Sao 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. (C) 2012 Elsevier Inc. All rights reserved.
Subject: Sickle cell disease
beta-Globin variant
Double-mutant hemoglobin
Hb S-Sao Paulo
Molecular dynamics simulation
Brazilian population
Editor: Elsevier
Citation: Archives of Biochemistry and Biophysics. Elsevier, v.519, n.1, p.23-31, 2012
Rights: fechado
Identifier DOI: 10.1016/
Date Issue: 2012
Appears in Collections:FCM - Artigos e Outros Documentos

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