Table 1 Summary of mutant mice.
From: NMJ-Analyser identifies subtle early changes in mouse models of neuromuscular disease
Strain/MGI | Mouse model | Background | Protein expression | Age (months) | Numbers/sex | Disease state | Ref |
|---|---|---|---|---|---|---|---|
SOD1G93A/+ MGI: 2448770 | Transgenic | C57BL/6N-SJL | ~ 20-fold overexpression | 1 | 5 WT, 5 mutants (male) | ALS, pre-symptomatic | |
1.5 | 5 WT, 5 mutants (male) | ALS, pre-symptomatic | |||||
3.5 | 4 WT, 9 mutants (male) | ALS, late symptomatic | |||||
GarsC201R/+ MGI: 3760297 | ENU mutagenesis | C57BL/6J | Physiological expression | 1 | 6 WT, 6 mutants (male) | CMT2D, early symptomatic | |
3 | 3 mutants (male) | CMT2D, symptomatic | |||||
FUSΔ14/+ MGI: 6100933 | Knock-in, partial humanization | C57BL/6J | Physiological expression | 3 | 4 WT, 4 mutants (male) | ALS, pre-symptomatic | |
12 | 4 WT, 4 mutants (male) | ALS, symptomatic | |||||
TDP43M323K/M323K MGI: 6355456 | ENU mutagenesis | C57BL/6J-DBA/2J | Physiological expression | 12 | 5 WT, 5 mutants (female) | ALS, pre-symptomatic |
