. "A variety of developmental disorders have been associated with ciliary defects, yet the controls that govern cilia disassembly are largely unknown. Here we report a mouse embryonic node gene, which we named Pitchfork (Pifo). Pifo associates with ciliary targeting complexes and accumulates at the basal body during cilia disassembly. Haploinsufficiency causes a unique node cilia duplication phenotype, left-right asymmetry defects, and heart failure. This phenotype is likely relevant in humans, because we identified a heterozygous R80K PIFO mutation in a fetus with situs inversus and cystic liver and kidneys, and in patient with double-outflow right ventricle. We show that PIFO, but not R80K PIFO, is sufficient to activate Aurora A, a protooncogenic kinase that induces cilia retraction, and that Pifo/PIFO mutation causes cilia retraction, basal body liberation, and overreplication defects. Thus, the observation of a disassembly phenotype in vivo provides an entry point to understand and categorize ciliary disease. AUTHOR AUDIO:" . . "Dev. Cell" . "Wurst W." . "Katsanis N." . "Ueffing M." . "Lickert H." . "Attie-Bitach T." . "Kinzel D." . "Davis E.E." . "Boldt K." . "Burtscher I." . "Trumbach D." . "Diplas B." . "2010"^^ . "66-77" . "Pitchfork regulates primary cilia disassembly and left-right asymmetry." . "19" . "doi:10.1016/j.devcel.2010.06.005" .