Diaphragm neuromuscular transmission failure in a mouse model of an early-onset neuromotor disorder
Matthew J. Fogarty, Joline E. Brandenburg, Gary C. Sieck
Abstract
Individuals with motor control deficits, including cerebral palsy (CP) often have respiratory impairments. Glycine-receptor mutant spa mice have early-onset hypertonia, and limb motor impairments, similar to individuals with CP. We hypothesized that in the diaphragm of spa mice, disruption of glycinergic inputs to MNs would result in increased phrenic–DIAm neuromuscular transmission failure. Pathophysiologic abnormalities in neuromuscular transmission may contribute to respiratory dysfunction in conditions where early developmental MN loss or motor control deficits are apparent.
Topics & Concepts
HypertoniaNeuromuscular transmissionCerebral palsyDiaphragm (acoustics)Glycine receptorRespiratory failurePathophysiologyNeuroscienceRespiratory systemMotor controlMedicineBulbar palsyPhysical medicine and rehabilitationPsychologyBiologyAnesthesiaInternal medicineGlycinePhysicsLoudspeakerAmino acidBiochemistryAcousticsNeurogenetic and Muscular Disorders ResearchCerebral Palsy and Movement DisordersNeuroscience of respiration and sleep