From: From animal models to human disease: a genetic approach for personalized medicine in ALS
Species | Number of repeats | Promoter | Age at onset (weeks) | Survival (weeks) | Phenotype | References | |||
---|---|---|---|---|---|---|---|---|---|
Paralysis | Cognitive symptoms | Neuropathological findings and particularities | Gliosis | ||||||
Mice | 80 | TRE | none | normal | N | N | ubiquitin-positive inclusion, no DPR, no TDP-43 inclusion | nd | [174] |
 | 66a | nd | 24 | nd | nd | Y | Nuclear RNA foci, phosphoTDP-43 inclusions, cytosolic and nuclear DPR, Anxiety and social abnormalities, motor impairment | Y | [176] |
 | 100–1000 | BACb | none | normal | N | N | RNA foci, DPR, no NCI | N | [178] |
 | 500 | BACb | none | normal | N | N | RNA foci, DPR, no NCI | N | [177] |
 | 450 | BACb | 52 | normal | N | Y | RNA foci, DPR, age-dependant protein accumulation, no motor deficits or MN loss, age dependant cognitive deficit, no TDP-43 mislocalization | N | [179] |
 | 500 | BACb | 16 | 20–40c | Y | Y | NMJ loss, reduced axonal size, MN loss, RNA foci, DPR, TDP-43 NCI | Y | [180] |
Fruit flies | 36–103 | elav-GS | nd | 30 days | nd | nd | RNA foci, DPR, toxicity was attributed to DPR | nd | [182] |
 | 160 | actin5C-Gal4 | none | normal | N | nd | RNA foci, DPR, toxicity was attributed to DPR | nd | [181] |
 | 30 | Ok371-Gal4 | 4 | nd | nd | nd | Decreased locomotor activity | nd | [183] |
 | 58 | Ok371-Gal4 | nd | nd | nd | nd | Decreased locomotor activity, NMJ loss, DPR | nd | [184] |
Nematodesd | n/a | alfa-1 | 2 | nd | Y | nd | MN loss, paralysis in 60Â % of worms | nd | [185] |
Zebrafishe | n/a | n/a | nd | nd | nd | nd | MN axons shortening, reduced swimming | nd | [186] |