Institute of Biomedical Sciences

Exploring 22q11.2 Deletion Syndrome in Murine Laryngeal Motor Neurons: An Electrophysiological Approach

Poster Number

22

Document Type

Poster

Status

Graduate Student - Doctoral

Abstract Category

Neuroscience

Keywords

Dysphagia, Swallowing, 22q11.2 Deletion Syndrome, Laryngeal Motor Neurons, Electrophysiology

Publication Date

Spring 2018

Abstract

Pediatric dysphagia, difficulty in swallowing and feeding, is one of the most common problems associated with various developmental disorders, and is especially prevalent in patients with 22q11.2 Deletion Syndrome (22q11DS). The act of swallowing is largely regulated through vagal innervation to the larynx from laryngeal motor neuron cell bodies located within the medullar portion of the brainstem. One laryngeal structure in particular, the epiglottis, is especially important as it works to seal off the trachea during active swallowing, preventing aspiration into the lungs. Using a large deletion (LD) mouse model for 22q11DS, the objective of this work aims to establish and characterize changes in the electrophysiological properties of laryngeal motor neurons that occur in LD animals. The spontaneous firing, evoked firing, voltage-gated currents, as well as both excitatory and inhibitory synaptic neurotransmission to laryngeal motor neurons recorded from the LD animals was quantified and compared to those from wild-type (WT) littermates. Preliminary data suggests that laryngeal neurons from LD animals possess a higher spontaneous action potential (AP) firing frequency than those from WT animals. Additionally, while excitatory postsynaptic currents (EPSCs) appear to occur at the same frequency in both LD and WT laryngeal motor neurons, the spontaneous excitatory events are reduced in amplitude in the LD neurons compared to those from the WT animals. Our overall goal is to determine the different electrophysiological properties of laryngeal neurons from LD animals compared to WT littermates. This work aims to provide the foundation needed to later identify therapeutic targets and hopefully develop novel treatments to restore laryngeal activity, as well as future possible treatment strategies for pediatric dysphagia associated with 22q11DS.

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Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.

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Exploring 22q11.2 Deletion Syndrome in Murine Laryngeal Motor Neurons: An Electrophysiological Approach

Pediatric dysphagia, difficulty in swallowing and feeding, is one of the most common problems associated with various developmental disorders, and is especially prevalent in patients with 22q11.2 Deletion Syndrome (22q11DS). The act of swallowing is largely regulated through vagal innervation to the larynx from laryngeal motor neuron cell bodies located within the medullar portion of the brainstem. One laryngeal structure in particular, the epiglottis, is especially important as it works to seal off the trachea during active swallowing, preventing aspiration into the lungs. Using a large deletion (LD) mouse model for 22q11DS, the objective of this work aims to establish and characterize changes in the electrophysiological properties of laryngeal motor neurons that occur in LD animals. The spontaneous firing, evoked firing, voltage-gated currents, as well as both excitatory and inhibitory synaptic neurotransmission to laryngeal motor neurons recorded from the LD animals was quantified and compared to those from wild-type (WT) littermates. Preliminary data suggests that laryngeal neurons from LD animals possess a higher spontaneous action potential (AP) firing frequency than those from WT animals. Additionally, while excitatory postsynaptic currents (EPSCs) appear to occur at the same frequency in both LD and WT laryngeal motor neurons, the spontaneous excitatory events are reduced in amplitude in the LD neurons compared to those from the WT animals. Our overall goal is to determine the different electrophysiological properties of laryngeal neurons from LD animals compared to WT littermates. This work aims to provide the foundation needed to later identify therapeutic targets and hopefully develop novel treatments to restore laryngeal activity, as well as future possible treatment strategies for pediatric dysphagia associated with 22q11DS.