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Front. Genet. | doi: 10.3389/fgene.2019.00154

The Trp73 mutant mice: a ciliopathy model that uncouples Ciliogenesis from Planar Cell Polarity

 Maria C. Marin1, 2*, Margarita M. Martinez3, Javier Villoch-Fernandez2, Laura Maeso-Alonso2 and  Sandra Fuertes-Alvarez2
  • 1Universidad de León, Spain
  • 2Laboratorio de Diferenciación Celular y Diseño de Modelos Celulares, Instituto de Biomedicina, Departamento de Biología Molecular, Spain
  • 3Institute of Livestock Development, University of León, Spain

p73 transcription factor belongs to one of the most important gene families in vertebrate biology, the p53-family. Trp73 gene, like the other family members, generates multiple isoforms named TA and DNp73, with different and, sometimes, antagonist functions. Although p73 shares many biological functions with p53, it also plays distinct roles during development. Trp73 null mice (p73KO from now on) show multiple phenotypes as gastrointestinal and cranial hemorrhages, rhinitis or central nervous system defects. Several groups, including ours, have revisited the apparently unrelated phenotypes observed in total p73KO and revealed a novel p73 function in the organization of ciliated epithelia in brain and trachea, but also an essential role as regulator of ependymal planar cell polarity. Unlike p73KO or TAp73KO mice, tumor-prone Trp53-/- mice do not present ependymal ciliary or planar cell polarity defects, indicating that regulation of ciliogenesis and PCP is a p73-specific function. Thus, loss of ciliary biogenesis and epithelial organization might be a common underlying cause of the diverse p73KO-phenotypes, highlighting Trp73 role as an architect of the epithelial tissue. In this review we would like to discuss the data regarding p73 role as regulator of ependymal cell ciliogenesis and PCP, supporting the view of the Trp73-mutant mice as a model that uncouples ciliogenesis from PCP and a possible model of human congenital hydrocephalus.

Keywords: TAp73, DNp73, ependymal cells, Ciliogenesis, Planar cell polarity (PCP), Microtubu]es, Actin Cytoskeleton, Hydrocefalus

Received: 20 Nov 2018; Accepted: 13 Feb 2019.

Edited by:

Jose M. Carvajal-Gonzalez, Universidad de Extremadura, Spain

Reviewed by:

David R. Kaplan, Hospital for Sick Children, Canada
NURIA M. MARIN, Gulbenkian Institute of Science, Portugal  

Copyright: © 2019 Marin, Martinez, Villoch-Fernandez, Maeso-Alonso and Fuertes-Alvarez. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Dr. Maria C. Marin, Universidad de León, León, Spain, carmen.marin@unileon.es