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

Table 1 Progressive phenotypic correlates in the G93A murine model of ALS1

					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

*V. Little evidence of vacuolar pathology at this late stage; **V. Little vacuolar pathology evident at end stage of phenotype.

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