A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS.
The SOD1-G93A transgenic mouse is the most widely used animal model of amyotrophic lateral sclerosis (ALS). At ALS TDI we developed a phenotypic screening protocol, demonstrated in video herein, which reliably assesses the neuromuscular function of SOD1-G93A mice in a quick manner. This protocol encompasses a simple neurological scoring system (NeuroScore) designed to assess hindlimb function. NeuroScore is focused on hindlimb function because hindlimb deficits are the earliest reported neurological sign of disease in SOD1-G93A mice. The protocol developed by ALS TDI provides an unbiased assessment of onset of paresis (slight or partial paralysis), progression and severity of paralysis and it is sensitive enough to identify drug-induced changes in disease progression. In this report, the combination of a detailed manuscript with video minimizes scoring ambiguities and inter-experimenter variability thus allowing for the protocol to be adopted by other laboratories and enabling comparisons between studies taking place at different settings. We believe that this video protocol can serve as an excellent training tool for present and future ALS researchers.
ALS Therapy Development Institute; thatzipetros@als.net.
Theo
T
Hatzipetros
ALS Therapy Development Institute.
Joshua D
JD
Kidd
ALS Therapy Development Institute.
Andy J
AJ
Moreno
ALS Therapy Development Institute.
Kenneth
K
Thompson
ALS Therapy Development Institute.
Alan
A
Gill
ALS Therapy Development Institute.
Fernando G
FG
Vieira
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D000818:Animals; D004195:Disease Models, Animal; D018450:Disease Progression; D005260:Female; D006614:Hindlimb / Q000503:physiopathology; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism
C470429:SOD1 G37R protein, mouse; D013482:Superoxide Dismutase