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Table 1 Progressive phenotypic correlates in the G93A 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 # of gene copies Relative SOD1 Protein Levels (brain) (ng SOD1 protein/μg tot soluble brain protein) Relative increase in SOD1 activity (U/μg soluble brain protein) Mean ALS onset (d) Mean age at death (d) Motor neuron vacuolation Motor neuron loss
Genomic hSOD1 G93A (G1) [5, 9, 13, 14] 18 2.2 2.56 90-120 153-185 73-163 >180
Genomic hSOD1 G93A (G1H) [5, 7, 15] 25 3.36 3.04 90 136 37 80
Genomic hSOD1 G93A (G1L) [16] 13 n/a n/a 200 251 200* 230
Genomic hSOD1 G93A (G5/G5) [7] 10 1.28 1.61 300 >400 ** >300
  1. *V. Little evidence of vacuolar pathology at this late stage; **V. Little vacuolar pathology evident at end stage of phenotype.