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

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

doi:https://doi.org/10.1016/j.abb.2012.01.001

Artículo

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

Jorge, Susan E. D. C.; Petruk, Ariel Alcides Icon

; 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

Fecha de publicación: 03/2012

Editorial: Elsevier Science Inc

Revista: Archives of Biochemistry and Biophysics

ISSN: 0003-9861

Idioma: Inglés

Tipo de recurso: Artículo publicado

Clasificación temática:

Biofísica

Resumen

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

Palabras clave: Β-Globin Variant , Brazilian Population , Double-Mutant Hemoglobin , Hb S-São Paulo , Molecular Dynamics Simulation , Sickle Cell Disease

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Excepto donde se diga explícitamente, este item se publica bajo la siguiente descripción: Creative Commons Attribution-NonCommercial-ShareAlike 2.5 Unported (CC BY-NC-SA 2.5)

Identificadores

URI: http://hdl.handle.net/11336/83673

URL: https://www.sciencedirect.com/science/article/pii/S0003986112000033

DOI: https://doi.org/10.1016/j.abb.2012.01.001

Colecciones

Articulos(INQUIMAE)
Articulos de INST.D/QUIM FIS D/L MATERIALES MEDIOAMB Y ENERGIA

Citación

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

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