Hb S-Sao Paulo: A new sickling hemoglobin with stable polymers and decreased oxygen affinity
| Contribuinte(s) |
UNIVERSIDADE DE SÃO PAULO |
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| Data(s) |
29/10/2013
29/10/2013
2012
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| 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. Sao Paulo Research Foundation (FAPESP) [2009/18248-3, 2008/57441-0] Sao Paulo Research Foundation (FAPESP) National Council for Research (CNPq) / Brazil National Council for Research (CNPq) / Brazil |
| Identificador |
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, NEW YORK, v. 519, n. 1, supl. 4, Part 1, pp. 23-31, 36951, 2012 0003-9861 http://www.producao.usp.br/handle/BDPI/36216 10.1016/j.abb.2012.01.001 |
| Idioma(s) |
eng |
| Publicador |
ELSEVIER SCIENCE INC NEW YORK |
| Relação |
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS |
| Direitos |
closedAccess Copyright ELSEVIER SCIENCE INC |
| Palavras-Chave | #SICKLE CELL DISEASE #BETA-GLOBIN VARIANT #DOUBLE-MUTANT HEMOGLOBIN #HB S-SAO PAULO #MOLECULAR DYNAMICS SIMULATION #BRAZILIAN POPULATION #MOLECULAR-DYNAMICS SIMULATIONS #AMINO-ACID SUBSTITUTIONS #BETA-POLYPEPTIDE-CHAIN #CELL-DISEASE #DEOXYHEMOGLOBIN-S #ALPHA-THALASSEMIA #2 RESIDUES #BINDING #VARIANT #DETERMINANTS #BIOCHEMISTRY & MOLECULAR BIOLOGY #BIOPHYSICS |
| Tipo |
article original article publishedVersion |
