PRICKLE1 encodes a nuclear receptor that may be a negative regulator of the Wnt/beta-catenin signaling pathway. The encoded protein localizes to the nuclear membrane and has been implicated in the nuclear trafficking of the transcription repressors REST/NRSF and REST4. Mutations in this gene have been linked to progressive myoclonus epilepsy. Alternate splicing results in multiple transcript variants. A pseudogene of this gene is found on chromosome 3. The deduced 831-amino acid protein contains 3 N-terminal LIM domains and 3 C-terminal nuclear localization signals. It also contains 4 N-glycosylation sites, 2 PKA phosphorylation sites, and a C-terminal CIIS (cys-ile-ile-ser) prenylation motif. Northern blot analysis detected a single 4.4-kb transcript in all tissues examined, with the highest level in placenta.