Figure 7.

Heterozygous HdhQ200 mice exhibit different patterns of progressive accumulation of striatal and cortical NIIs and AF. (A) NIIs were labeled with an N-terminal htt antibody. NIIs are apparent as early as 20 weeks and become more robust and abundant by 40 weeks in both striatum and cortex. (B) HdhQ200 heterozygous mice exhibit progressive AF. AF were expressed diffusely in striatal neurons as early as 9 weeks and expressed robustly at 20 weeks. No AF are detected in WT mice at any age. At 40 weeks, AF appear distinctly punctate and perinuclear (denoted by arrows). (A and B) Heterogeneous populations of mhtt aggregates exist in different subcellular compartments, one cytoplasmic (AF) and the other nuclear (NIIs). (C) In heterozygous HdhQ200 striata at 40 weeks, perinuclear compartmentalization of punctuate AF was confirmed by confocal microscopy in which AF were labeled with Alexa Fluorophore 488 (green) and nuclei with DAPI (blue).