Figure 2.

NAC Suppresses Aggregation of Mutant Ataxin-3

(A) Filter trap aggregation assay of full-length Ataxin-3 containing a stretch of 78 glutamines (AtxQ78) incubated with 1× molar excess of NAC at 37°C for the indicated time. SDS-insoluble aggregates were detected with an anti-His antibody.

(B) Quantification of SDS-insoluble AtxQ78 aggregates obtained in filter trap from (A). Data are representative of at least 3 independent biological replicates.

(C) NAC and AtxQ78 were incubated in a 1:1 molar ratio and crosslinked using the homobifunctional crosslinker BS³. The schematic shows all intermolecular BS³ crosslinks identified by MS analysis of NAC-AtxQ78 complexes excised from the gel shown in Figure S2C.

See also FigureS2 and Table S1.