FUZ (fuzzy planar cell polarity protein) is a core component of the CPLANE complex that plays essential roles in ciliogenesis and planar cell polarity signaling 1. As part of the CPLANE complex, FUZ works alongside INTU, WDPCP, JBTS17, and RSG1 to regulate cilium assembly and maintenance 1. The protein functions as a probable GEF (guanine nucleotide exchange factor) for Rab GTPases through its homologous Longin domains, facilitating vesicular trafficking during ciliogenesis 2. FUZ regulates Sonic Hedgehog signaling by controlling ciliary localization of GPR161, a negative regulator of the pathway, demonstrating genetic epistasis between these proteins 3. Clinically, FUZ mutations cause skeletal ciliopathies including short-rib polydactyly syndrome type IV, characterized by skeletal malformations and polydactyly 4. During embryonic development, FUZ is critical for proper neural tube formation, with knockout mice exhibiting exencephaly, spina bifida, and hypoplastic hindbrain development with abnormal rhombomere morphology 5. Additionally, FUZ dysregulation is implicated in neurodegeneration and cancer, where it can trigger apoptosis through dishevelled/Rac1/JNK signaling and shows reduced expression in certain cancers associated with poor prognosis 67.