3-Pyridinecarboximidoyl chloride, N-[2-hydroxy-3-(1-piperidinyl)propoxy]-, 1-oxide

In Anti-neurodegeneration agent 1 (Compound I)-treated mSODl^(G93A) mice, Western blot analysis reveals an observable 'band shift' of HSF-I, indicative of a stress-induced activation of HSF-I by hyper-phosphorylation. Immunostaining reveals that, at 120 days of age, expression of Hsp70 and Hsp90 is increased in the lumbar spinal cords of both untreated and Anti-neurodegeneration agent 1- treated SOD1^(G93A) mice, although there is a clear increase in the intensity of Hsp70 and Hsp90 immunoreactivity in motor neurons of Anti-neurodegeneration agent 1-treated mSODl^(G93A) mice. Anti-neurodegeneration agent I- treated mice live an average of 153 days (±2.6 SEM, n=7). This represents a significant increase in lifespan of over 22% (p=<0.001). The effect of beginning Anti-neurodegeneration agent 1 treatment at the time of disease onset is also tested by starting treatment at 70 days of age, when the first signs of locomotor defects are observed. Anti-neurodegeneration agent 1 treatment from 70 days of age extended the mean lifespan of mSODl^(G93A) mice by 23 days, from 125 days (±1.8 SEM, n=18) in the untreated group to 148 days (±1.5 SEM, n=5) in the treated group. This represents an increase in lifespan of 18% (p=<0.001)^[1]