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  CPAP promotes timely cilium disassembly to maintain neural progenitor pool.

Gabriel, E., Wason, A., Ramani, A., Gooi, L. M., Keller, P., Pozniakovsky, A. I., et al. (2016). CPAP promotes timely cilium disassembly to maintain neural progenitor pool. The EMBO Journal, 35(8), 803-819.

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 Creators:
Gabriel, Elke, Author
Wason, Arpit, Author
Ramani, Anand, Author
Gooi, Li Ming, Author
Keller, Patrick1, Author           
Pozniakovsky, Andrei I.1, Author           
Poser, Ina1, Author           
Noack, Florian2, Author
Telugu, Narasimha Swamy, Author
Calegari, Federico3, Author           
Šarić, Tomo, Author
Hescheler, Juergen, Author
Hyman, Anthony1, Author           
Gottardo, Marco, Author
Callaini, Giuliano, Author
Alkuraya, Fowzan Sami, Author
Gopalakrishnan, Jay3, Author           
Affiliations:
1Max Planck Institute of Molecular Cell Biology and Genetics, Max Planck Society, ou_2340692              
2Max Planck Society, ou_persistent13              
3External Organizations, ou_persistent22              

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 Abstract: A mutation in the centrosomal-P4.1-associated protein (CPAP) causes Seckel syndrome with microcephaly, which is suggested to arise from a decline in neural progenitor cells (NPCs) during development. However, mechanisms ofNPCs maintenance remain unclear. Here, we report an unexpected role for the cilium inNPCs maintenance and identifyCPAPas a negative regulator of ciliary length independent of its role in centrosome biogenesis. At the onset of cilium disassembly,CPAPprovides a scaffold for the cilium disassembly complex (CDC), which includes Nde1, Aurora A, andOFD1, recruited to the ciliary base for timely cilium disassembly. In contrast, mutatedCPAPfails to localize at the ciliary base associated with inefficientCDCrecruitment, long cilia, retarded cilium disassembly, and delayed cell cycle re-entry leading to premature differentiation of patientiPS-derivedNPCs. AberrantCDCfunction also promotes premature differentiation ofNPCs in SeckeliPS-derived organoids. Thus, our results suggest a role for cilia in microcephaly and its involvement during neurogenesis and brain size control.

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 Dates: 2016
 Publication Status: Issued
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 Identifiers: eDoc: 732427
Other: 6460
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Title: The EMBO Journal
Source Genre: Journal
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Pages: - Volume / Issue: 35 (8) Sequence Number: - Start / End Page: 803 - 819 Identifier: -