Loss of SHH pathway inhibition in neural stem cells and GCs from lack of GPR161 and SUFU, which promotes cleavage of GLI3 into the transcriptional repressor GLI3R, can lead to hyperproliferation and cerebellar dysplasia (Blaess et al., 2008; Jiwani et al., 2020; Shimada et al., 2018). Here, SUFU is linked to Cerebellar dysplasia.