. Author manuscript; available in PMC: 2022 Apr 1.

Published in final edited form as: J Physiol. 2021 Jan 9;599(7):2037–2054. doi: 10.1113/JP280978

Table 1: Comparison of Thap1^+/− mutant mice with other mouse models of dystonia.

Summary of cerebellar neuron dysfunction across mouse models of dystonia with phenotypic manifestations showing that low firing frequency is present in all models with reporter tremor, whereas reduced firing regularity is observed in all mouse models manifesting with dystonia-like postures.

Mouse model:	Thap1^+/−	Ptf1a^Cre/+; Vglut2^fl/fl	TorsinA KD	Sgce KD	ATP1α3 KD	Cerebellar ouabain
Dystonia type:	DYT6	−	DYT1	DYT11	DYT12	DYT12
PC firing	F –; R ↑	F –; R –	F ↓; R ↓	F –; R ↓	F –; R –	F ↑; R ↓
CN firing	F ↓; R –	F ↓; R ↓	F ↓; R ↓	F ↓; R ↓	F ↑; R ↓	F ↑; R ↓
Dystonia	N	Y	Y	Y	Y	Y
Tremor	Y	Y	NR	NR	NR	NR
Gait	N	Y	Y	Y	Y	Y

KD= RNA Knock Down; F= firing frequency; R= firing regularity; N= not observed; Y= observed; NR= not reported.

Table 1: Comparison of Thap1+/− mutant mice with other mouse models of dystonia.

Table 1: Comparison of Thap1^+/− mutant mice with other mouse models of dystonia.