Table 1.

Inhibitors of GSK3β with a positive effect in the pre-clinical studies of HSA^LR mice.

GSK3 Inhibitor	Effect on the HSALR Phenotype	Molecular Effects
Lithium [31]	◾Reduction of myopathy in adult mice ◾Reduction of myotonia in adult mice ◾Reduction of the grip weakness in adult mice	◾Normalization of GSK3β ◾Normalization of cyclin D3 ◾Normalization of CUGBP1 activity
TDZD-8 [31]	◾Reduction of myotonia in adult mice ◾Reduction of the grip weakness in adult mice ◾Activation of myogenic satellite cells in adult muscle	◾Normalization of GSK3β ◾Normalization of cyclin D3 ◾Increase of PAX-7
BIO [49]	◾Reduction of myopathy in adult mice ◾Reduction of the grip weakness in adult mice ◾Prevention of muscle pathology (myopathy and the grip weakness) in adult mice after short treatment at a young age (6 weeks)	◾Normalization of GSKβ ◾Normalization of cyclin D3 ◾Normalization of CUGBP1 levels ◾Normalization of CUGBP1 activity ◾Increase of PAX-7 ◾Normalization of myogenic downstream targets of CUGBP1: RBM45, LEF1, DCX and Col 4A
Tideglusib [50]	◾Reduction of myopathy in adult mice ◾Reduction of the grip weakness in adult mice	◾Reduction of the mutant CUG repeats ◾Reduction of CUG foci ◾Normalization of GSK3β ◾Normalization of myogenic downstream targets of CUGBP1, DCX and RMB45 ◾Normalization of splicing (Serca1, Cypher)