SHH and Meckel syndrome, type 1: In the present study, we demonstrate that loss of the ciliary Frizzled-like receptor TMEM67, which is mutated in the ciliopathies MKS and JBTS, causes a severe cerebellar hypoplasia phenotype due to complex Wnt signalling, ciliogenesis and rostral hindbrain patterning defects that impact on downstream Shh signalling events.