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dc.contributor.author
Jorge, Susan E. D. C.
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Petruk, Ariel Alcides
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Kimura, Elza M.
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Oliveira, Denise M.
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Caire, Lucas
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Suemasu, Cintia N.
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Silveira, Paulo A. A.
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Albuquerque, Dulcineia M.
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Costa, Fernando F.
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Skaf, Munir S.
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Martínez, Leandro
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De Fatima Sonati, Maria
dc.date.available
2019-09-16T20:57:38Z
dc.date.issued
2012-03
dc.identifier.citation
Jorge, Susan E. D. C.; Petruk, Ariel Alcides; Kimura, Elza M.; Oliveira, Denise M.; Caire, Lucas; et al.; Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity; Elsevier Science Inc; Archives of Biochemistry and Biophysics; 519; 1; 3-2012; 23-31
dc.identifier.issn
0003-9861
dc.identifier.uri
http://hdl.handle.net/11336/83673
dc.description.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
dc.format
application/pdf
dc.language.iso
eng
dc.publisher
Elsevier Science Inc
dc.rights
info:eu-repo/semantics/openAccess
dc.rights.uri
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.subject
Β-Globin Variant
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Brazilian Population
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Double-Mutant Hemoglobin
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Hb S-São Paulo
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Molecular Dynamics Simulation
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Sickle Cell Disease
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Biofísica
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Ciencias Biológicas
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CIENCIAS NATURALES Y EXACTAS
dc.title
Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
dc.type
info:eu-repo/semantics/article
dc.type
info:ar-repo/semantics/artículo
dc.type
info:eu-repo/semantics/publishedVersion
dc.date.updated
2019-06-11T18:17:17Z
dc.journal.volume
519
dc.journal.number
1
dc.journal.pagination
23-31
dc.journal.pais
Países Bajos
dc.journal.ciudad
Amsterdam
dc.description.fil
Fil: Jorge, Susan E. D. C.. Universidade Estadual de Campinas; Brasil
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Fil: Petruk, Ariel Alcides. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Tucumán. Instituto Superior de Investigaciones Biológicas. Universidad Nacional de Tucumán. Instituto Superior de Investigaciones Biológicas; Argentina
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Fil: Kimura, Elza M.. Universidade Estadual de Campinas; Brasil
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Fil: Oliveira, Denise M.. Universidade Estadual de Campinas; Brasil
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Fil: Caire, Lucas. Universidade Estadual de Campinas; Brasil
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Fil: Suemasu, Cintia N.. Universidade Estadual de Campinas; Brasil
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Fil: Silveira, Paulo A. A.. Albert Einstein Institute; Brasil
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Fil: Albuquerque, Dulcineia M.. Universidade Estadual de Campinas; Brasil
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Fil: Costa, Fernando F.. Universidade Estadual de Campinas; Brasil
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Fil: Skaf, Munir S.. Universidade Estadual de Campinas; Brasil
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Fil: Martínez, Leandro. Universidade de Sao Paulo; Brasil
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Fil: De Fatima Sonati, Maria. Universidade Estadual de Campinas; Brasil
dc.journal.title
Archives of Biochemistry and Biophysics
dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S0003986112000033
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info:eu-repo/semantics/altIdentifier/doi/https://doi.org/10.1016/j.abb.2012.01.001
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