ArticleProceedings of the National Academy of Sciences of the United States of America2013
Role of p63 and the Notch pathway in cochlea development and sensorineural deafness.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 39 citations in OpenAlex.
- The ubiquitin-proteasome system and proteostasis in the cochlea and sensorineural hearing disorders.Molecular biomedicine · 2026Review
- Safety considerations for cereblon-recruiting targeted protein degraders.Nature reviews. Drug discovery · 2026Review
- Deep Phenotyping and Molecular Elucidation of a New Syndrome: Ectodermal Dysplasia Caused by IRF6 Variants.Experimental dermatology · 2026Article
- BRD4 sustains p63 transcriptional program in keratinocytes.Biology direct · 2024Article
- ΔNp63α-mediated epigenetic regulation in keratinocyte senescence.Epigenetics · 2023Article
- Inhibition of TGF-β signaling enables long-term proliferation of mouse primary epithelial stem/progenitor cells of the tympanic membrane and the middle ear mucosa.Scientific reports · 2023Article
- Differential genetic diagnoses of adult post-lingual hearing loss according to the audiogram pattern and novel candidate gene evaluation.Human genetics · 2022Article
- The miR-133a, TPM4 and TAp63γ Role in Myocyte Differentiation Microfilament Remodelling and Colon Cancer Progression.International journal of molecular sciences · 2021Article
- TGF-β1 Facilitates TAp63α Protein Lysosomal Degradation to Promote Pancreatic Cancer Cell Migration.Biology · 2021Article
- Molecular Mechanisms of the Teratogenic Effects of Thalidomide.Pharmaceuticals (Basel, Switzerland) · 2020Review
- Molecular mechanisms of thalidomide and its derivatives.Proceedings of the Japan Academy. Series B, Physical and biological sciences · 2020Article
- p63 is a cereblon substrate involved in thalidomide teratogenicity.Nature chemical biology · 2019Article
- Ectrodactyly-ectodermal dysplasia-clefting syndrome with unusual cutaneous vitiligoid and psoriasiform lesions due to a novel single pointPostepy dermatologii i alergologii · 2019Article
- Novel missense and 3'-UTR splice site variants in LHFPL5 cause autosomal recessive nonsyndromic hearing impairment.Journal of human genetics · 2018Article
- Spatiotemporal coordination of cellular differentiation and tissue morphogenesis in organ of Corti development.Medical molecular morphology · 2018Review
- Master regulatory role of p63 in epidermal development and disease.Cellular and molecular life sciences : CMLS · 2018Review
- Transcriptional regulation of P63 on the apoptosis of male germ cells and three stages of spermatogenesis in mice.Cell death & disease · 2018Article
- Characterization of the Transcriptomes of Lgr5+ Hair Cell Progenitors and Lgr5- Supporting Cells in the Mouse Cochlea.Frontiers in molecular neuroscience · 2017Article
- NOTCH3 Is Induced in Cancer-Associated Fibroblasts and Promotes Angiogenesis in Oral Squamous Cell Carcinoma.PloS one · 2016Article
- OTX2 regulates the expression of TAp63 leading to macular and cochlear neuroepithelium development.Aging · 2015Article
Corrections and comments
- Erratum issued
Authors and funding
10 authors at 7 institutions in 5 countries.
Funding
Abstract
The ectodermal dysplasias are a group of inherited autosomal dominant syndromes associated with heterozygous mutations in the Tumor Protein p63 (TRP63) gene. Here we show that, in addition to their epidermal pathology, a proportion of these patients have distinct levels of deafness. Accordingly, p63 null mouse embryos show marked cochlea abnormalities, and the transactivating isoform of p63 (TAp63) protein is normally found in the organ of Corti. TAp63 transactivates hairy and enhancer of split 5 (Hes5) and atonal homolog 1 (Atoh1), components of the Notch pathway, known to be involved in cochlear neuroepithelial development. Strikingly, p63 null mice show morphological defects of the organ of Corti, with supernumerary hair cells, as also reported for Hes5 null mice. This phenotype is related to loss of a differentiation property of TAp63 and not to loss of its proapoptotic function, because cochleas in mice lacking the critical Bcl-2 homology domain (BH-3) inducers of p53- and p63-mediated apoptosis--Puma, Noxa, or both--are normal. Collectively, these data demonstrate that TAp63, acting via the Notch pathway, is crucial for the development of the organ of Corti, providing a molecular explanation for the sensorineural deafness in ectodermal dysplasia patients with TRP63 mutations.
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