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

dc.creatorJorge, Susan E. D. C.
dc.creatorPetruk, Ariel A.
dc.creatorKimura, Elza M.
dc.creatorOliveira, Denise M.
dc.creatorCaire, Lucas
dc.creatorSuemasu, Cintia N.
dc.creatorSilveira, Paulo A. A.
dc.creatorAlbuquerque, Dulcineia M.
dc.creatorCosta, Fernando F.
dc.creatorSkaf, Munir S.
dc.creatorMartínez, Leandro
dc.creatorSonati, Maria de Fatima
dc.date.accessioned2013-10-29T12:27:57Z
dc.date.accessioned2018-07-04T16:02:09Z
dc.date.available2013-10-29T12:27:57Z
dc.date.available2018-07-04T16:02:09Z
dc.date.created2013-10-29T12:27:57Z
dc.date.issued2012
dc.identifierARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, NEW YORK, v. 519, n. 1, supl. 4, Part 1, pp. 23-31, 36951, 2012
dc.identifier0003-9861
dc.identifierhttp://www.producao.usp.br/handle/BDPI/36216
dc.identifier10.1016/j.abb.2012.01.001
dc.identifierhttp://dx.doi.org/10.1016/j.abb.2012.01.001
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1630738
dc.description.abstractHb 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.
dc.languageeng
dc.publisherELSEVIER SCIENCE INC
dc.publisherNEW YORK
dc.relationARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS
dc.rightsCopyright ELSEVIER SCIENCE INC
dc.rightsclosedAccess
dc.subjectSICKLE CELL DISEASE
dc.subjectBETA-GLOBIN VARIANT
dc.subjectDOUBLE-MUTANT HEMOGLOBIN
dc.subjectHB S-SAO PAULO
dc.subjectMOLECULAR DYNAMICS SIMULATION
dc.subjectBRAZILIAN POPULATION
dc.titleHb S-Sao Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
dc.typeArtículos de revistas


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