Changes in structure and function of diaphragm neuromuscular junctions from BACHD mouse model for Huntington's disease

Neurochemistry International
Bárbara Campos de AragãoC Guatimosim

Abstract

Huntington's disease (HD) is a neurodegenerative disorder characterized by a progressive decline of motor and cognitive functions. It is caused by a polyglutamine expansion in the huntingtin (htt) protein, which then leads to neurodegeneration that span both the central and peripheral nervous system. Previous works have shown that htt interacts with several proteins from the neurotransmitter release machinery causing synaptic dysfunction. In this work, we looked for alterations in diaphragm neuromuscular junctions (NMJs) from 3 to 4 months old BACHD mouse model for HD. This model represents a new and robust in vivo paradigm for studying the pathogenesis of HD. For optical analysis, NMJs were stained with FM1-43fx and α-bungarotoxin to visualize both pre and postsynaptic elements, respectively. Confocal microscopy optical analysis showed a decrease in the number of synaptic elements and fluorescence intensity in NMJs from BACHD diaphragms compared to WT. We next analyzed presynaptic activity and we observed that synaptic vesicle exocytosis was impaired in NMJs from BACHD diaphragms. Ultrastructural analysis revealed significant changes in the form and sizes of the synaptic vesicles in BACHD diaphragm NMJs that could contribute t...Continue Reading

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Citations

Jun 4, 2017·Physiological Reports·Tamara S CutlerChristopher S Colwell
Dec 3, 2016·The Journal of General Physiology·Daniel R MirandaAndrew A Voss
Nov 23, 2017·Frontiers in Behavioral Neuroscience·Giuseppe ManfréHuu Phuc Nguyen
Nov 11, 2020·International Journal of Molecular Sciences·Manuela Bozzi, Francesca Sciandra
Dec 18, 2020·Neuropeptides·Julliane V Joviano-SantosCristina Guatimosim

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