Summary of Pre-clinical Mouse Models of ATP1A3-related Disorders.
Summary of Pre-clinical Mouse Models of ATP1A3-related Disorders.
| Model | Mouse Genome Informatics | Phenotype | Spontaneous Seizures | Reference |
|---|---|---|---|---|
| ATP1A3tm/1Ling/+ (Alpha3+/KOI4) | 3696954 | Motor hyperactivity, mania-like behavior, sleep cycle abnormalities | No | Kirshenbaum et al. (2011)99 |
| ATP1A3tm2Kwk | 5572809 | Motor hyperactivity, no gross morphological defects or apparent histological brain anomalies, no development of dystonia spontaneously | No | Ikeda et al. (2013)100 |
| ATP1A3D801Y (alpha3D801Y; ATP1A3tm1Klh) | 6163502 | Hyperactivity, increased sensitivity to chemically induced epileptic seizures, and cognitive deficits | No | Holm and Lykke-Hartmann (2016)101 |
| Myshkin (ATP1A3Myk) | 4356167 | Motor hyperactivity, spontaneous and induced by vestibular stress, hippocampal hyperexcitation, memory and learning reduction | Yes | Clapcote et al. (2009)102
Kirshenbaum et al. (2011)99 |
| Mashlool (Mashl; ATP1A3tm1Ute) | 6162645 | Small size, hyperactivity, ataxia and tremor, hind-limb clasping (tail suspension), reversible hemiplegia, quadriplegia, and dystonia, avoidant and anxiety-like behaviors | Yes | Hunanyan et al. (2015)4 |
| Matoub (Matb; ATP1A3E815K; ATP1A3tm1Mika) | 6197036 | Small size, hemiplegia/dystonia/seizures spontaneously and upon stimulation, abnormal episodic memory, abnormal motor performance and coordination, less activity and freezing; high mortality | Yes | Helseth et al. (2018)103 |
| ROSA26-ATP1A3D591V [Gt(ROSA) 26Sortm1(CAG-ATP1A3-D591V,-EGFP)] | NA | Scarce description in the literature regarding the phenotype | No | Zhou et al. (2020)104 |