Results

Here we report that WTxCCS mice die early. We then use the WTxCCS mice to investigate how different compounds get Cu in to the CNS.
 Homozygous human CCS transgenic mice (described in Williams et al., 2016) were crossed with hemizygous human SODWT transgenic mice (B6SJL-Tg(SOD1)2Gur/J) to produce SODWTxCCS and NTGxCCS offspring.  Unlike previous studies, which reported SODWTxCCS mice to have no abnormal phenotype (Son et al., 2008), here we report that our SODWTxCCS mice consistently experience an early development crisis similar to but less severe than high-expressing SODG93AxCCS mice. Our SODWTxCCS mice rapidly developed paralysis and died within three weeks (Figure 1). In six years of breeding, this result has been consistent.