Transgenic Mouse Model for ALS

SOD1^G37R/YFP (SOD1^+/-; YFP^+/-) transgenic mice were previously described (Martineau et al., 2018 (link)). Briefly, they were obtained by breeding male loxSOD1^G37R mice (RRID:IMSR_JAX:016149) with heterozygote female Thy1-YFP, line H, mice (B6.Cg-Tg(Thy1-YFP)HJrs/J; The Jackson Laboratory, stock number 003782; RRID:IMSR_JAX:003782). Thy1-YFP mice from line H were used due to their sparse expression of the yellow-fluorescent protein (YFP) in lower motor neurons (Feng et al., 2000 (link)) allowing visualization of single motor axons. Parent lines were maintained on a C57BL6/J background. Importantly, transmission of both transgenes follows a Mendelian autosomal pattern of inheritance, ruling out the possibility that either transgene is integrated on a sex chromosome. Disease progression and motor function were monitored via weekly weight and grip strength measurements (BioSeb, BIO-GS3). Animals were sacrificed using a lethal dose of isoflurane. All experiments were performed in accordance with the guidelines of the Canadian Council on Animal Care, the Comité de Déontologie sur l’Expérimentation Animale of Université de Montréal (protocol #18–040), and the CRCHUM Institutional Committee for the Protection of Animals (protocol #N16008CVV and #N15047ADPs).