A method comparison in monitoring disease progression of G93A mouse model of ALS.
Our aim was to find an optimal method for monitoring disease progression and assessing the effectiveness of new agents in SOD1 mice. We compared six testing methods including clinical grading, weighing, hanging wire test, rotarod test, motor neuron counting and motor unit number estimation (MUNE) in SOD1 mice and control animals. The six methods were all able to differentiate between control animals and SOD1 transgenic mice at some time points; of them motor neuron counting, weighing and MUNE could detect abnormalities in presymptomatic stage in SOD1 mice; The number of functional motor units precisely correlated with motor neuron counts (r = 0.894, p<0.01). We concluded that MUNE is the better testing measure for monitoring disease and evaluating pharmacological therapies in SOD1 mice, which not only can reflect a meaningful biological effect of the agents used, but also is a sensitive, accurate and practical method.
Zhou
Chang
C
Department of Neurology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong, PR China.
Zhao
Cui-Ping
CP
Zhang
Chen
C
Wu
Guo-Yong
GY
Xiong
Fu
F
Zhang
Cheng
C
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*; D000818:Animals; D002452:Cell Count / Q000379:methods; D004195:Disease Models, Animal; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000473:pathology; D011999:Recruitment, Neurophysiological; D045442:Rotarod Performance Test / Q000379:methods; D013482:Superoxide Dismutase / Q000235:genetics*
C109415:SOD1 G93A protein; D013482:Superoxide Dismutase