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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