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

Autor(es):Mostrar menos -

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]

Afiliação do(s) autor(es)

^[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

• Tipo de documento: Artigo Científico
• Fonte: Archives of Biochemistry and Biophysics; v. 519, n. 1, p. 23-31, MAR 1 2012.
• Citações Web of Science: 2

Resumo

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)

• Processo FAPESP: 09/18248-3 - Correlação Estrutura-Função de Variantes Estruturais da Hemoglobina Humana
• Beneficiário: Susan Elisabeth Domingues Costa Jorge
• Modalidade de apoio: Bolsas no Brasil - Doutorado
• Processo FAPESP: 08/57441-0 - Alterações clínicas, celulares e moleculares nas hemoglobinopatias e em outras anemias hemolíticas hereditárias
• Beneficiário: Fernando Ferreira Costa
• Modalidade de apoio: Auxílio à Pesquisa - Temático