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(SOD1G93A)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(SOD1G93A)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-TARDBPQ331K)103Dwc/J (017933) mice, also called Prp-TDP-43Q331K line 103, express a mutant human TAR DNA binding protein associated with familial ALS. The Prp-TDP-43Q331K [~1.5x] mice have moderate overexpression levels in total TDP-43 mRNA/protein, with an ~2.5-fold increase in total TDP-43 expression (huTDP-43Q331K levels ~1.5-fold greater) compared to endogenous TDP-43 in non-transgenic mice. By three months of age, Prp-TDP-43Q331K [~1.5x] mice develop adult-onset motor dysfunction (as measured by rotarod performance) accompanied by a loss of hindlimb-grip strength and the appearance of muscle fasciculations.