B6SJL-TgN[SOD1-G93A]1Gur mice29,  which show Amyotrophic Lateral Sclerosis (ALS) related clinical symptoms starting at  about 14 -15 weeks of age, presenting as weakness progressing into disability to ambulate, with loss of lumbar motor neurons, and death at 18-20 weeks.

References

Gotkine M, Rozenstein L, Einstein O, Abramsky O, Argov Z, Rosenmann H (2013) Presymptomatic treatment with acetylcholinesterase antisense oligonucleotides prolongs survival in ALS G93A-SOD1) mice. BioMed Research International 2013:845345