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

Autores
Jorge, Susan E. D. C.; Petruk, Ariel Alcides; Kimura, Elza M.; Oliveira, Denise M.; Caire, Lucas; Suemasu, Cintia N.; Silveira, Paulo A. A.; Albuquerque, Dulcineia M.; Costa, Fernando F.; Skaf, Munir S.; Martínez, Leandro; De Fatima Sonati, Maria
Año de publicación
2012
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
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
Fil: Jorge, Susan E. D. C.. Universidade Estadual de Campinas; Brasil
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
Fil: Kimura, Elza M.. Universidade Estadual de Campinas; Brasil
Fil: Oliveira, Denise M.. Universidade Estadual de Campinas; Brasil
Fil: Caire, Lucas. Universidade Estadual de Campinas; Brasil
Fil: Suemasu, Cintia N.. Universidade Estadual de Campinas; Brasil
Fil: Silveira, Paulo A. A.. Albert Einstein Institute; Brasil
Fil: Albuquerque, Dulcineia M.. Universidade Estadual de Campinas; Brasil
Fil: Costa, Fernando F.. Universidade Estadual de Campinas; Brasil
Fil: Skaf, Munir S.. Universidade Estadual de Campinas; Brasil
Fil: Martínez, Leandro. Universidade de Sao Paulo; Brasil
Fil: De Fatima Sonati, Maria. Universidade Estadual de Campinas; Brasil
Materia
Β-Globin Variant
Brazilian Population
Double-Mutant Hemoglobin
Hb S-São Paulo
Molecular Dynamics Simulation
Sickle Cell Disease
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/83673
Acceder
id CONICETDig_a5541eae3be15af43caae76cd25f6236
oai_identifier_str oai:ri.conicet.gov.ar:11336/83673
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinityJorge, Susan E. D. C.Petruk, Ariel AlcidesKimura, Elza M.Oliveira, Denise M.Caire, LucasSuemasu, Cintia N.Silveira, Paulo A. A.Albuquerque, Dulcineia M.Costa, Fernando F.Skaf, Munir S.Martínez, LeandroDe Fatima Sonati, MariaΒ-Globin VariantBrazilian PopulationDouble-Mutant HemoglobinHb S-São PauloMolecular Dynamics SimulationSickle Cell Diseasehttps://purl.org/becyt/ford/1.6https://purl.org/becyt/ford/1Hb 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 observationsFil: Jorge, Susan E. D. C.. Universidade Estadual de Campinas; BrasilFil: 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; ArgentinaFil: Kimura, Elza M.. Universidade Estadual de Campinas; BrasilFil: Oliveira, Denise M.. Universidade Estadual de Campinas; BrasilFil: Caire, Lucas. Universidade Estadual de Campinas; BrasilFil: Suemasu, Cintia N.. Universidade Estadual de Campinas; BrasilFil: Silveira, Paulo A. A.. Albert Einstein Institute; BrasilFil: Albuquerque, Dulcineia M.. Universidade Estadual de Campinas; BrasilFil: Costa, Fernando F.. Universidade Estadual de Campinas; BrasilFil: Skaf, Munir S.. Universidade Estadual de Campinas; BrasilFil: Martínez, Leandro. Universidade de Sao Paulo; BrasilFil: De Fatima Sonati, Maria. Universidade Estadual de Campinas; BrasilElsevier Science Inc2012-03info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/83673Jorge, 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; 23-310003-9861CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S0003986112000033info:eu-repo/semantics/altIdentifier/doi/10.1016/j.abb.2012.01.001info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-10-22T11:04:10Zoai:ri.conicet.gov.ar:11336/83673instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-10-22 11:04:10.231CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
title Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
spellingShingle Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
Jorge, Susan E. D. C.
Β-Globin Variant
Brazilian Population
Double-Mutant Hemoglobin
Hb S-São Paulo
Molecular Dynamics Simulation
Sickle Cell Disease
title_short Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
title_full Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
title_fullStr Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
title_full_unstemmed Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
title_sort Hb S-São Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
dc.creator.none.fl_str_mv Jorge, Susan E. D. C.
Petruk, Ariel Alcides
Kimura, Elza M.
Oliveira, Denise M.
Caire, Lucas
Suemasu, Cintia N.
Silveira, Paulo A. A.
Albuquerque, Dulcineia M.
Costa, Fernando F.
Skaf, Munir S.
Martínez, Leandro
De Fatima Sonati, Maria
author Jorge, Susan E. D. C.
author_facet Jorge, Susan E. D. C.
Petruk, Ariel Alcides
Kimura, Elza M.
Oliveira, Denise M.
Caire, Lucas
Suemasu, Cintia N.
Silveira, Paulo A. A.
Albuquerque, Dulcineia M.
Costa, Fernando F.
Skaf, Munir S.
Martínez, Leandro
De Fatima Sonati, Maria
author_role author
author2 Petruk, Ariel Alcides
Kimura, Elza M.
Oliveira, Denise M.
Caire, Lucas
Suemasu, Cintia N.
Silveira, Paulo A. A.
Albuquerque, Dulcineia M.
Costa, Fernando F.
Skaf, Munir S.
Martínez, Leandro
De Fatima Sonati, Maria
author2_role author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Β-Globin Variant
Brazilian Population
Double-Mutant Hemoglobin
Hb S-São Paulo
Molecular Dynamics Simulation
Sickle Cell Disease
topic Β-Globin Variant
Brazilian Population
Double-Mutant Hemoglobin
Hb S-São Paulo
Molecular Dynamics Simulation
Sickle Cell Disease
purl_subject.fl_str_mv https://purl.org/becyt/ford/1.6
https://purl.org/becyt/ford/1
dc.description.none.fl_txt_mv 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
Fil: Jorge, Susan E. D. C.. Universidade Estadual de Campinas; Brasil
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
Fil: Kimura, Elza M.. Universidade Estadual de Campinas; Brasil
Fil: Oliveira, Denise M.. Universidade Estadual de Campinas; Brasil
Fil: Caire, Lucas. Universidade Estadual de Campinas; Brasil
Fil: Suemasu, Cintia N.. Universidade Estadual de Campinas; Brasil
Fil: Silveira, Paulo A. A.. Albert Einstein Institute; Brasil
Fil: Albuquerque, Dulcineia M.. Universidade Estadual de Campinas; Brasil
Fil: Costa, Fernando F.. Universidade Estadual de Campinas; Brasil
Fil: Skaf, Munir S.. Universidade Estadual de Campinas; Brasil
Fil: Martínez, Leandro. Universidade de Sao Paulo; Brasil
Fil: De Fatima Sonati, Maria. Universidade Estadual de Campinas; Brasil
description 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
publishDate 2012
dc.date.none.fl_str_mv 2012-03
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/83673
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
0003-9861
CONICET Digital
CONICET
url http://hdl.handle.net/11336/83673
identifier_str_mv 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
0003-9861
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://www.sciencedirect.com/science/article/pii/S0003986112000033
info:eu-repo/semantics/altIdentifier/doi/10.1016/j.abb.2012.01.001
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Elsevier Science Inc
publisher.none.fl_str_mv Elsevier Science Inc
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
_version_ 1846781291974885376
score 12.982451

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