AI Article Synopsis
- A new type of hemoglobin called Hb S-São Paulo was identified in an 18-month-old Brazilian boy, who had moderate anemia, carrying two specific mutations.
- Hb S-São Paulo exhibits similar properties to Hb S in certain tests but shows distinct behaviors in others, particularly in alkaline conditions and advanced chromatography techniques.
- The variant hemoglobin is slightly unstable and has a lower oxygen-binding ability, and it tends to form more stable polymers than Hb S, which may be influenced by electrostatic interactions.
Article Abstract
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.
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| http://dx.doi.org/10.1016/j.abb.2012.01.001 | DOI Listing |
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