| Title: | Loss of p73 in ependymal cells during the perinatal period leads to aqueductal stenosis |
| Author(s): | Fujitani M; Sato R; Yamashita T; |
| Address: | "Department of Molecular Neuroscience, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan. fujitani@hyo-med.ac.jp. Molecular Research Center for Children's Mental Development, United Graduate School of Child Development, Osaka University, 2-2 Yamadaoka, Suita, Osaka, 565-0872, Japan. fujitani@hyo-med.ac.jp. Department of Anatomy and Neuroscience, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663-8501, Japan. fujitani@hyo-med.ac.jp. Department of Molecular Neuroscience, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan. Department of Molecular Neuroscience, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan. yamashita@molneu.med.osaka-u.ac.jp. World Premier International, Immunology Frontier Research Center, Osaka University, 3-1 Yamadaoka, Suita-shi, Osaka, 565-0871, Japan. yamashita@molneu.med.osaka-u.ac.jp. Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamadaoka, Suita-shi, Osaka, 565-0871, Japan. yamashita@molneu.med.osaka-u.ac.jp" |
| DOI: | 10.1038/s41598-017-12105-z |
| ISSN/ISBN: | 2045-2322 (Electronic) 2045-2322 (Linking) |
| Abstract: | "The p53 family member p73 plays a critical role in brain development. p73 knockout mice exhibit a number of deficits in the nervous system, such as neuronal death, hydrocephalus, hippocampal dysgenesis, and pheromonal defects. Among these phenotypes, the mechanisms of hydrocephalus remain unknown. In this study, we generated a p73 knock-in (KI) mutant mouse and a conditional p73 knockout mouse. The homozygous KI mutants showed aqueductal stenosis. p73 was expressed in the ependymal cell layer and several brain areas. Unexpectedly, when p73 was disrupted during the postnatal period, animals showed aqueductal stenosis at a later stage but not hydrocephalus. An assessment of the integrity of cilia and basal body (BB) patch formation suggests that p73 is required to establish translational polarity but not to establish rotational polarity or the planar polarization of BB patches. Deletion of p73 in adult ependymal cells did not affect the maintenance of translational polarity. These results suggest that the loss of p73 during the embryonic period is critical for hydrocephalus development" |
| Keywords: | "Animals Brain/cytology/embryology/*metabolism Cell Polarity/genetics Cilia/genetics/metabolism Ependyma/cytology/embryology/*metabolism Gene Expression Regulation, Developmental Hydrocephalus/genetics/*metabolism/pathology Mice, Knockout Mice, Transgenic;neuroscience;" |
| Notes: | "MedlineFujitani, Masashi Sato, Ryohei Yamashita, Toshihide eng Research Support, Non-U.S. Gov't England 2017/09/22 Sci Rep. 2017 Sep 20; 7(1):12007. doi: 10.1038/s41598-017-12105-z" |
