deLorimier et al. (2017) revealed a clear connection between pseudouridine and neuronal abnormalities in patients with type 2 myotonic dystrophy. They demonstrated that pseudouridine decreases the binding of MBNL1 at extended CCUG repeats, which is responsible for causing illness (Angelova et al., 2018; deLorimier et al., 2017). Here, MBNL1 is linked to Proximal myotonic myopathy.