ALS Efficacy Studies
Efficacy studies performed by JAX® In Vivo Services for ALS typically feature clinical observations, body weights, progressive neurological scoring, electrophysiological assessment of motor neuropathy, survival, tissue/blood collection and histological assessment of neuromuscular junctions and femoral nerves.
- B6SJL-Tg(SOD1*G93A)1Gur/J (002726), also called G93A-SOD1, express a human transgene that carries the G93A mutant form of human SOD1 on a mixed C57BL/6J and SJL/J genetic background.
- B6.Cg-Tg(SOD1*G93A)1Gur/J (004435) mice carry the same transgene as 002726 but on a C57BL/6J congenic background. They develop similar phenotypes as the mixed background strain, but have a slightly longer life span.
- B6.Cg-Tg(Prnp- TARDBP*A315T)95Balo/J (010700) mice, also called Prp-TDP43A315T, express a mutant human TAR DNA binding protein associated with familial ALS. Hemizygous mice develop a progressive and fatal disease associated with neurodegeneration of the myenteric plexus and exhibit cellular brain pathology reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates.