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Persistent Feeding and Swallowing Deficits in a Mouse Model of 22q11.2 Deletion Syndrome

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dc.contributor.authorWelby, Laurenen
dc.contributor.authorCaudill, Haileyen
dc.contributor.authorYitsege, Gelilaen
dc.contributor.authorHamad, Alien
dc.contributor.authorBunyak, Filizen
dc.contributor.authorZohn, Irene E.en
dc.contributor.authorMaynard, Thomas M.en
dc.contributor.authorLaMantia, Anthony-Samuelen
dc.contributor.authorMendelowitz, Daviden
dc.contributor.authorLever, Teresa E.en
dc.contributor.departmentBiological Sciencesen
dc.contributor.departmentFralin Biomedical Research Instituteen
dc.contributor.departmentVirginia Tech Carilion School of Medicineen
dc.date.accessioned2020-05-20T17:01:09Zen
dc.date.available2020-05-20T17:01:09Zen
dc.date.issued2020-01-31en
dc.description.abstractDisrupted development of oropharyngeal structures as well as cranial nerve and brainstem circuits may lead to feeding and swallowing difficulties in children with 22q11. 2 deletion syndrome (22q11DS). We previously demonstrated aspiration-based dysphagia during early postnatal life in the LgDel mouse model of 22q11DS along with disrupted oropharyngeal morphogenesis and divergent differentiation and function of cranial motor and sensory nerves. We now ask whether feeding and swallowing deficits persist in adult LgDel mice using methods analogous to those used in human patients to evaluate feeding and swallowing dysfunction. Compared to wild-type mice, videofluoroscopic swallow study revealed that LgDel mice have altered feeding and swallowing behaviors, including slower lick rates, longer inter-lick intervals, and longer pharyngeal transit times with liquid consistency. Transoral endoscopic assessment identified minor structural anomalies of the palate and larynx in one-third of the LgDel mice examined. Video surveillance of feeding-related behaviors showed that LgDel mice eat and drink more frequently. Furthermore, LgDel animals engage in another oromotor behavior, grooming, more frequently, implying that divergent craniofacial and cranial nerve structure and function result in altered oromotor coordination. Finally, LgDel mice have significantly increased lung inflammation, a potential sign of aspiration-based dysphagia, consistent with results from our previous studies of early postnatal animals showing aspiration-related lung inflammation. Thus, oromotor dysfunction, feeding, and swallowing difficulties and their consequences persist in the LgDel 22q11DS mouse model. Apparently, postnatal growth and/or neural plasticity does not fully resolve deficits due to anomalous hindbrain, craniofacial, and cranial nerve development that prefigure perinatal dysphagia in 22q11DS. This new recognition of persistent challenges with feeding and swallowing may provide opportunities for improved therapeutic intervention for adolescents and adults with 22q11DS, as well as others with a history of perinatal feeding and swallowing disorders.en
dc.description.notesThe behavioral and craniofacial analysis for this work were supported by the Eunice Kennedy Shriver National Institute of Child Health & Human Development of the National Institutes of Health (5P01HD083157). The microscopic analysis for this study was conducted at the CRI Light Microscopy and Image Analysis Core, supported by the Intellectual and Developmental Disabilities Research Center Award (U54HD090257) through the Eunice Kennedy Shriver National Institute of Child Health and Human Development of the National Institutes of Health.en
dc.description.sponsorshipEunice Kennedy Shriver National Institute of Child Health & Human Development of the National Institutes of Health [5P01HD083157]; Intellectual and Developmental Disabilities Research Center Award through the Eunice Kennedy Shriver National Institute of Child Health and Human Development of the National Institutes of Health [U54HD090257]en
dc.format.mimetypeapplication/pdfen
dc.identifier.doihttps://doi.org/10.3389/fneur.2020.00004en
dc.identifier.issn1664-2295en
dc.identifier.other4en
dc.identifier.pmid32082240en
dc.identifier.urihttp://hdl.handle.net/10919/98500en
dc.identifier.volume11en
dc.language.isoenen
dc.rightsCreative Commons Attribution 4.0 Internationalen
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.subject22q11 deletion syndromeen
dc.subjectDiGeorge syndromeen
dc.subjectpediatric dysphagiaen
dc.subjectdysphagiaen
dc.subjectdeglutitionen
dc.subjectfeedingen
dc.subjectmouse modelen
dc.titlePersistent Feeding and Swallowing Deficits in a Mouse Model of 22q11.2 Deletion Syndromeen
dc.title.serialFrontiers in Neurologyen
dc.typeArticle - Refereeden
dc.type.dcmitypeTexten
dc.type.dcmitypeStillImageen

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