CPAP promotes timely cilium disassembly to maintain neural progenitor pool

• DOI: 10.15252/embj.201593679
• Publication Date: 2016-03-01T03:23:40Z
• Authors: Elke Gabriel; Arpit Wason; Anand Ramani; Li Ming Gooi; Patrick Keller; Andrei Pozniakovsky; Ina Poser; Florian Noack; Narasimha Swamy T...; Federico Calegari; Tomo Šarić; Jürgen Hescheler; Anthony A Hyman; Marco Gottardo; Giuliano Callaini; Fowzan Sami Alkuraya; Jay Gopalakrishnan
• Keywords: Intraflagellar Transport; Microcephaly; Ciliopathies; Progenitor

Abstract A mutation in the centrosomal‐P4.1‐associated protein ( CPAP ) causes Seckel syndrome with microcephaly, which is suggested to arise from a decline neural progenitor cells NPC s) during development. However, mechanisms of s maintenance remain unclear. Here, we report an unexpected role for cilium and identify as negative regulator ciliary length independent its centrosome biogenesis. At onset disassembly, provides scaffold disassembly complex CDC ), includes Nde1, Aurora A, OFD 1, recruited base timely disassembly. In contrast, mutated fails localize at associated inefficient recruitment, long cilia, retarded delayed cell cycle re‐entry leading premature differentiation patient iPS ‐derived s. Aberrant function also promotes organoids. Thus, our results suggest cilia microcephaly involvement neurogenesis brain size control.