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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Author(s):
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Jorge, Susan E. D. C. [1] ; Petruk, Ariel A. [2] ; Kimura, Elza M. [1] ; Oliveira, Denise M. [1] ; Caire, Lucas [3] ; Suemasu, Cintia N. [1] ; Silveira, Paulo A. A. [4] ; Albuquerque, Dulcineia M. [5] ; Costa, Fernando F. [5] ; Skaf, Munir S. [3] ; Martinez, Leandro [6] ; Sonati, Maria de Fatima [1]
Total Authors: 12
Affiliation:
[1] State Univ Campinas UNICAMP, Sch Med Sci, Dept Clin Pathol, BR-13083970 Campinas, SP - Brazil
[2] Inst Super Invest Biol CONICET UNT, San Miguel De Tucuman - Argentina
[3] State Univ Campinas UNICAMP, Inst Chem, BR-13083970 Campinas, SP - Brazil
[4] Albert Einstein Inst, Sao Paulo - Brazil
[5] State Univ Campinas UNICAMP, Hematol & Hemotherapy Ctr, BR-13083970 Campinas, SP - Brazil
[6] Univ Sao Paulo, Sao Carlos Inst Phys, Sao Carlos, SP - Brazil
Total Affiliations: 6
Document type: Journal article
Source: Archives of Biochemistry and Biophysics; v. 519, n. 1, p. 23-31, MAR 1 2012.
Web of Science Citations: 2
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. (AU)

FAPESP's process: 08/57441-0 - Clinical, cellular and molecular alterations in hemoglobinopathies and other hereditary hemolytic anemias
Grantee:Fernando Ferreira Costa
Support type: Research Projects - Thematic Grants
FAPESP's process: 09/18248-3 - Correlation between function and structure of structural variants of the human hemoglobin
Grantee:Susan Elisabeth Domingues Costa Jorge
Support type: Scholarships in Brazil - Doctorate