Direct measurement of
prefibrillar HbS oligomers by DLS and FRET.
(A) Confirmation of oligomerization via DLS. Purified HbA or HbS (25
μM) was vacuum degassed with argon for 5 min, and then DLS measurements
were taken at room temperature. (B) HbS oligomer size versus concentration.
Purified HbS (0.01–100 μM) was degassed with argon for
5 min, and then DLS measurements were taken at room temperature. (C)
Schematic of TR-FRET scheme. Time-resolved FRET can be used to probe
association of deoxy-HbS monomers in solution and to discover small
molecules that disrupt these assemblies. (D) FRET efficiency of fluorescent
dyes, fluorescently labeled HbA and HbS samples under deoxygenated
and oxygenated conditions. FRET efficiency of HbS sample was increased
under deoxygenated conditions, demonstrating that the signal is specific
to association of HbS in the absence of fibers. Voxelotor and phenylalanine,
which are known to inhibit polymerization, reduced FRET efficiency
of HbS under deoxygenated conditions. All DLS data are reported as
the effective diameter of the mean assembly size. Hypothesis testing
was performed using parametric ANOVA with Dunnett’s multiple
comparison testing. *p-value < 0.05; **p-value < 0.01; ***p-value < 0.001.