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Disease Models & Mechanisms
- Source :
- Disease Models & Mechanisms, article-version (VoR) Version of Record
- Publication Year :
- 2021
- Publisher :
- The Company of Biologists, 2021.
-
Abstract
- 22q11.2 Deletion Syndrome (22q11DS) is a neurodevelopmental disorder associated with cranial nerve anomalies and disordered oropharyngeal function, including pediatric dysphagia. Using the LgDel 22q11DS mouse model, we investigated whether sensory neuron differentiation in the trigeminal ganglion (CNgV), which is essential for normal orofacial function, is disrupted. We did not detect changes in cranial placode cell translocation or neural crest migration at early stages of LgDel CNgV development. However, as the ganglion coalesces, proportions of placode-derived LgDel CNgV cells increase relative to neural crest cells. In addition, local aggregation of placode-derived cells increases and aggregation of neural crest-derived cells decreases in LgDel CNgV. This change in cell-cell relationships was accompanied by altered proliferation of placode-derived cells at embryonic day (E)9.5, and premature neurogenesis from neural crest-derived precursors, reflected by an increased frequency of asymmetric neurogenic divisions for neural crest-derived precursors by E10.5. These early differences in LgDel CNgV genesis prefigure changes in sensory neuron differentiation and gene expression by postnatal day 8, when early signs of cranial nerve dysfunction associated with pediatric dysphagia are observed in LgDel mice. Apparently, 22q11 deletion destabilizes CNgV sensory neuron genesis and differentiation by increasing variability in cell-cell interaction, proliferation and sensory neuron differentiation. This early developmental divergence and its consequences may contribute to oropharyngeal dysfunction, including suckling, feeding and swallowing disruptions at birth, and additional orofacial sensory/motor deficits throughout life.<br />Summary: Altered proportions, distribution, timing and modes of division for trigeminal ganglion progenitors prefigure divergent trigeminal sensory neuron differentiation and oropharyngeal dysfunction in the LgDel mouse model of 22q11.2 deletion syndrome.
- Subjects :
- Cranial places
Sensory neurons
Sensory Receptor Cells
Neurogenesis
Precursor proliferation
Neuroscience (miscellaneous)
Medicine (miscellaneous)
Sensory system
Biology
General Biochemistry, Genetics and Molecular Biology
Mice
Neural crest
Trigeminal ganglion
Neurodevelopmental disorder
22q11 Deletion Syndrome
DMM ras
Immunology and Microbiology (miscellaneous)
DiGeorge Syndrome
medicine
Animals
Humans
22q11 deletion syndrome
Cell Differentiation
medicine.disease
Sensory neuron
Ganglion
medicine.anatomical_structure
Neuroscience
Research Article
Subjects
Details
- ISSN :
- 17548411 and 17548403
- Volume :
- 15
- Database :
- OpenAIRE
- Journal :
- Disease Models & Mechanisms
- Accession number :
- edsair.doi.dedup.....746c46b3b0a7da6644ce0fd298c46469