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Table 6 FUS animal models

From: From animal models to human disease: a genetic approach for personalized medicine in ALS

Species Mutation Promoter Age at onset (days) Survival (days) Phenotype References
Paralysis Cognitive symptoms Neuropathological findings and particularities Gliosis
Mice KO n/a nd nd nd nd 24 h death, chromosomal abnormality, sterility nd [198]
  KO n/a nd nd nd Y No motor phenotype, hyperactivity behavior nd [199]
  hFUSWT mPrp 4 weeks 10–13 weeks Y N Tremors, weight loss, deficit in rotarod, increase cytoplasmic FUS signal, spinal MN loss, NMJ loss Y [200]
  hFUSWT, R521G CAG 10 30 Na Y More lethality in FUSWT, no MN loss in lateral column, MA, NMJ loss, reduced social interaction and motor performance Y [201]
Rats hFUSWT, R521Cb TRE 27–48 33–55 Y Y Few MN loss, MA, NMJ loss, ubiquitin inclusions, no FUS inclusions Y [202]
Fruit flies hFUSWT, R524S, P525L OK371-Gal4 nd nd nd nd Large MN, decreased locomotor function, NMJ loss nd [203]
  hFUSWT, R518K, R521C, R521H OK371-Gal4/elav-GS 10 ~17 nd nd Decreased locomotor function, no NMJ loss, more cytosolic FUS in mutant, nd [204]
Nematodes hFUSWT, R514G, R521G, R522G, P525L, FUS513, FUS501 Prgef-1 3 8.1–9.7 Y nd NCI of mutant FUS, worst phenotype in R522G, P525L, FUS513 and FUS501 nd [206]
  S57Δ unc-47 12–13 normal Y nd MN loss, FUS insoluble aggregates nd [207]
Zebrafish R521C, R521H, S57Δ mRNA 48 hpf nd nd nd Reduced swimming at TEER, NMJ loss nd [208, 209]
  1. hFUS WT rats develop cognitive symptom at one year of age, Y yes, N no, nd not described, n/a not applicable, MN motor neuron, NMJ neuromuscular junction, MA muscle atrophy, NCI neuronal cytosolic inclusion, TEER touch-evoked escape response, hpf hours post-fertilization
  2. aReduced grip strength and hindlimb clasping
  3. bOnly hFUSR521C rats develop paralysis and features of ALS