ArticleScientific reports2017
Requirement for Jagged1-Notch2 signaling in patterning the bones of the mouse and human middle ear.
Article in Scientific reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 41 citations in OpenAlex.
- Phenotypic Divergence of JAG1- and NOTCH2-Associated Alagille Syndrome & Disease-Specific NOTCH2 Variant Classification Guidelines.Liver international : official journal of the International Association for the Study of the Liver · 2025Article
- A Retrospective Study of Clinical and Genetic Features in a Long-Term Cohort of Mexican Children with Alagille Syndrome.International journal of molecular sciences · 2025Article
- Nkx2.7 is a conserved regulator of craniofacial development.Nature communications · 2025Article
- The Gq/11 family of Gα subunits is necessary and sufficient for lower jaw development.Development (Cambridge, England) · 2025Article
- Jag1 represses Notch activation in lateral supporting cells and inhibits an outer hair cell fate in the medial cochlea.Development (Cambridge, England) · 2024Article
- The Effects of Viral Infections on the Molecular and Signaling Pathways Involved in the Development of the PAOs.Viruses · 2024Review
- Endothelin signaling in development.Development (Cambridge, England) · 2023Review
- Alagille Syndrome: A Case Report.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2022Article
- Article
- Notch signaling: Its essential roles in bone and craniofacial development.Genes & diseases · 2021Review
- Functional Diversification of SRSF Protein Kinase to Control Ubiquitin-Dependent Neurodevelopmental Signaling.Developmental cell · 2020Article
- Uncovering the secreted signals and transcription factors regulating the development of mammalian middle ear ossicles.Developmental dynamics : an official publication of the American Association of Anatomists · 2020Review
- Outcomes of Childhood Cholestasis in Alagille Syndrome: Results of a Multicenter Observational Study.Hepatology communications · 2020Article
- Article
- Growth factor and receptor malfunctions associated with human genetic deafness.Clinical genetics · 2020Review
- Evolution and development of the fish jaw skeleton.Wiley interdisciplinary reviews. Developmental biology · 2019Review
- Altered bone growth dynamics prefigure craniosynostosis in a zebrafish model of Saethre-Chotzen syndrome.eLife · 2018Article
- Essential Role of Nr2f Nuclear Receptors in Patterning the Vertebrate Upper Jaw.Developmental cell · 2018Article
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Authors and funding
10 authors at 2 institutions in 2 countries.
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Abstract
Whereas Jagged1-Notch2 signaling is known to pattern the sensorineural components of the inner ear, its role in middle ear development has been less clear. We previously reported a role for Jagged-Notch signaling in shaping skeletal elements derived from the first two pharyngeal arches of zebrafish. Here we show a conserved requirement for Jagged1-Notch2 signaling in patterning the stapes and incus middle ear bones derived from the equivalent pharyngeal arches of mammals. Mice lacking Jagged1 or Notch2 in neural crest-derived cells (NCCs) of the pharyngeal arches display a malformed stapes. Heterozygous Jagged1 knockout mice, a model for Alagille Syndrome (AGS), also display stapes and incus defects. We find that Jagged1-Notch2 signaling functions early to pattern the stapes cartilage template, with stapes malformations correlating with hearing loss across all frequencies. We observe similar stapes defects and hearing loss in one patient with heterozygous JAGGED1 loss, and a diversity of conductive and sensorineural hearing loss in nearly half of AGS patients, many of which carry JAGGED1 mutations. Our findings reveal deep conservation of Jagged1-Notch2 signaling in patterning the pharyngeal arches from fish to mouse to man, despite the very different functions of their skeletal derivatives in jaw support and sound transduction.
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