Table 2.

Inhibitors of GSK3 with a positive effect on the phenotype of DMSXL mice, that contain the human DMPK gene with more than 1000 CTG repeats in the preclinical studies.

GSK3 Inhibitor	Effect on the Phenotype of DMSXL Mice	Molecular Effects
Tideglusib [50]	◾Improvement of muscle histopathology in adult mice ◾Increase of survival after prenatal or postnatal treatment ◾Increase of postnatal growth after prenatal treatment ◾Increase of the grip strength after prenatal treatment ◾Reduction of anxiety after prenatal treatment	◾Normalization of GSK3β in skeletal muscle and in brain ◾Normalization of cyclin D3 in skeletal muscle ◾Increase of Pax-7 ◾Restoring CUGBP1 activity in brain ◾Normalization of CUGBP1 downstream targets in brain, Rbm45, MBNL3, Smn1 and Fgf-2