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    Transcriptional dysregulation in developing trigeminal sensory neurons in the LgDel mouse model of DiGeorge 22q11.2 deletion syndrome

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    Author
    Maynard, T.M.
    Horvath, A.
    Fraser, C.M.
    Date
    2020
    Journal
    Human molecular genetics
    Publisher
    Oxford University Press.
    Type
    Article
    
    Metadata
    Show full item record
    See at
    https://doi.org/10.1093/hmg/ddaa024
    Abstract
    LgDel mice, which model the heterozygous deletion of genes at human chromosome 22q11.2 associated with DiGeorge/22q11.2 deletion syndrome (22q11DS), have cranial nerve and craniofacial dysfunction as well as disrupted suckling, feeding and swallowing, similar to key 22q11DS phenotypes. Divergent trigeminal nerve (CN V) differentiation and altered trigeminal ganglion (CNgV) cellular composition prefigure these disruptions in LgDel embryos. We therefore asked whether a distinct transcriptional state in a specific population of early differentiating LgDel cranial sensory neurons, those in CNgV, a major source of innervation for appropriate oropharyngeal function, underlies this departure from typical development. LgDel versus wild-type (WT) CNgV transcriptomes differ significantly at E10.5 just after the ganglion has coalesced. Some changes parallel altered proportions of cranial placode versus cranial neural crest-derived CNgV cells. Others are consistent with a shift in anterior-posterior patterning associated with divergent LgDel cranial nerve differentiation. The most robust quantitative distinction, however, is statistically verifiable increased variability of expression levels for most of the over 17?000 genes expressed in common in LgDel versus WT CNgV. Thus, quantitative expression changes of functionally relevant genes and increased stochastic variation across the entire CNgV transcriptome at the onset of CN V differentiation prefigure subsequent disruption of cranial nerve differentiation and oropharyngeal function in LgDel mice. Copyright The Author(s) 2020. Published by Oxford University Press.
    Keyword
    LgDel mice embryo
    trigeminal sensory neurons
    Phenotype
    DiGeorge Syndrome
    Transcription, Genetic
    Neural Crest
    Cranial Nerves
    Sensory Receptor Cells
    Identifier to cite or link to this item
    https://www.scopus.com/inward/record.uri?eid=2-s2.0-85083544260&doi=10.1093%2fhmg%2fddaa024&partnerID=40&md5=c5ea7f75824a84aafadf55da98e38ca3; http://hdl.handle.net/10713/12668
    ae974a485f413a2113503eed53cd6c53
    10.1093/hmg/ddaa024
    Scopus Count
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    UMB Open Access Articles 2020

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