Edward J. Smith

This study develops and evaluates two novel antibodies, 1B12 and 11G2, for more sensitive detection of HTT1a in Huntington's disease mouse models compared to the existing MW8 antibody.

This study compares allele-selective and non-allele-selective antisense oligonucleotides in HD mice, showing that targeting mutant HTT intron-1 reduces HTT1a and improves disease outcomes.

The paper shows that lowering HTT1a transcript levels delays molecular and neuropathological phenotypes in a Huntington's disease mouse model.

This study shows that reducing MSH3 in Huntington's disease mouse models prevents somatic CAG repeat expansion but does not accelerate disease onset, suggesting somatic expansion may not be required for pathogenesis.

This study uses correlative light and electron microscopy to show that mutant huntingtin accumulates in endolysosomal organelles in presymptomatic Huntington's disease, altering endolysosomal homeostasis.