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. 2018 Oct 9;8:587. doi: 10.7916/D89S37MV

Table 3. Genetic Animal Models of Tremor.

Gene/Lesion Tremor Type (Hz) Tremor Measure Ataxia/Others Cerebellar Pathology/Physiology Reference
Mouse Car8 mutation Tremor (4–14 Hz) Tremor monitor (San Diego instruments) Ataxia Microzonal organization defects, abnormal Purkinje cell firing 53
crv4 mutation Intention tremor Observation Ataxia 83
Cp/Heph mutation Tremor Observation Ataxia 84
D801N mutation Tremor Observation Abnormal motor coordination 85
GABAA α1 subunit knockout Tremor (15–19 Hz) Tremor measured by suspending the tail and attached to the stereo speaker Absent spontaneous GABAergic inhibitory postsynaptic potentials 66
NPC1 mutation Tremor Observation Motor impairment, hyperactivity, impaired learning and memory 86
SCN8A mutation Tremor Observation Ataxia and dystonia Impaired repetitive firing of Purkinje cells in cerebellar slices 87
SOD1 mutation Tremor Observation Loss of extension reflex in hind-limbs, decreased grip strength and paralysis 88
SULT4A1 mutation Tremor Observation Ataxia and absence seizures 89
Vglut2 deletion in the climbing fiber synapses Tremor (4–14 Hz) Tremor monitor (San Diego instruments) Dystonia Abnormal Purkinje cell simple spike firing (Silencing climbing fiber synaptic transmission) 90
Wdr81 mutation Tremor Observation Abnormal gait Purkinje cell degeneration 91
β-III spectrin knockout Tremor Observation Motor incoordination and a wide hindlimb gait Purkinje cell loss and cerebellar atrophy 92
Fig4 knockout Intention tremor Observation hypomyelination of the cerebellum and spongiform degeneration in the deep cerebellar nuclei 93
Pura knockout Action tremor Observation Waddling gait Reduced number of Purkinje cells and granule cells 94
Sticky mouse (Aars mutation) Tremor Observation Ataxia Purkinje cell degeneration 95
Scrambler mouse Body tremor Observation Abnormal gait 96
Toppler mouse Action tremor Observation Ataxia Purkinje cell loss 97
Wobbler mouse Head tremor Observation Unsteady gait and muscle atrophy 98
Weaver mouse Tremor Observation Ataxia and hypertonia 99
Rat VF mutation Generalized tremor (especially the caudal body) that peaks between 4–8 weeks and gradually subsides Observation Abnormal myelin-associated vacuoles in the white matter of cerebellum 100
Shaker mutation Tremor (4–5 Hz) Force plate-based measurement Ataxia Purkinje cell degeneration 60
TRM/Kyo mutation Whole body tremor, responsive to propranolol Observation 102
Hamster bt mutation Tremor Observation Defective myelination in the central nervous system 101