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Table 2 Progressive phenotypic correlates in the G37R murine model of ALS1

From: Reduction in hSOD1 copy number significantly impacts ALS phenotype presentation in G37R (line 29) mice: implications for the assessment of putative therapeutic agents

  Age at pathology (d)
Genetic modification Relative SOD1 protein levels (human/mouse) spinal cord Total increase in SOD1 activity ALS onset (d) Motor neuron vacuolation Motor neuron loss Reactive astrogliosis
Genomic hSOD1 G37R(42) [10] 12.3 14.5 106-121 77 105 77
Genomic hSOD1 G37R(9) [10] 6.2 9 152-183 70 105 35
Genomic hSOD1 G37R(106) [10] 5.3 7.2 167-228 189 126 77
Genomic hSOD1 G37R(29) [10] 5 7 183-243 none 133 77
Genomic hSOD1 wild-type (76) [10] 10.5 9.2 None by 548d none none none