RT info:eu-repo/semantics/article
T1 The Trp73 Mutant Mice: A Ciliopathy Model That Uncouples Ciliogenesis From Planar Cell Polarity
A1 Marqués Martínez, Margarita
A1 Villoch-Fernández, Javier
A1 Maeso Alonso, Laura
A1 Fuertes Álvarez, Sandra
A1 Marín Vieira, María Carmen
A2 Biologia Celular
K1 Biología
K1 Genética
K1 TAp73
K1 DNp73
K1 Ependymal cells
K1 Ciliogenesis
K1 Planar cell polarity
K1 Microtubules
K1 Actin cytoskeleton
K1 Hydrocephalus
K1 2407 Biología Celular
K1 2407.02 Citogenética
AB [EN] 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 and severe 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 (p53KO) 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
PB Frontiers Media
LK https://hdl.handle.net/10612/20312
UL https://hdl.handle.net/10612/20312
NO Marques, M. M., Villoch-Fernandez, J., Maeso-Alonso, L., Fuertes-Alvarez, S. and Marin, M. C. (2019). The Trp73 mutant mice: a ciliopathy model that uncouples ciliogenesis from planar cell polarity. Frontiers in Genetics, 10, Article e154. https://doi.org/10.3389/fgene.2019.00154
NO Sec. Genetics of Common and Rare Diseases
DS BULERIA. Repositorio Institucional de la Universidad de León
RD 02-jun-2024