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Circuit-Specific Early Impairment of Proprioceptive Sensory Neurons in the SOD1(G93A) Mouse Model for ALS
Univ Calif Los Angeles, CA 90095 USA.
Univ Calif Los Angeles, CA 90095 USA.
Univ Calif Los Angeles, CA 90095 USA.
Univ Calif Los Angeles, CA 90095 USA; Univ Calif Los Angeles, CA 90095 USA.
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2019 (English)In: Journal of Neuroscience, ISSN 0270-6474, E-ISSN 1529-2401, Vol. 39, no 44, p. 8798-8815Article in journal (Refereed) Published
Abstract [en]

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease in which motor neurons degenerate, resulting in muscle atrophy, paralysis, and fatality. Studies using mouse models of ALS indicate a protracted period of disease development with progressive motor neuron pathology, evident as early as embryonic and postnatal stages. Key missing information includes concomitant alterations in the sensorimotor circuit essential for normal development and function of the neuromuscular system. Leveraging unique brainstem circuitry, we show in vitro evidence for reflex circuit-specific postnatal abnormalities in the jaw proprioceptive sensory neurons in the well-studied SOD1(G)(93A) mouse. These include impaired and arrhythmic action potential burst discharge associated with a deficit in Nav 1.6 Na+ channels. However, the mechanoreceptive and nociceptive trigeminal ganglion neurons and the visual sensory retinal ganglion neurons were resistant to excitability changes in age-matched SOD1(G)(93A )mice. Computational modeling of the observed disruption in sensory patterns predicted asynchronous self-sustained motor neuron discharge suggestive of imminent reflexive defects, such as muscle fasciculations in ALS. These results demonstrate a novel reflex circuit-specific proprioceptive sensory abnormality in ALS.

Place, publisher, year, edition, pages
SOC NEUROSCIENCE , 2019. Vol. 39, no 44, p. 8798-8815
Keywords [en]
amyotrophic lateral sclerosis; dynamic clamp; motor neurons; persistent sodium; proprioceptive neurons; SOD1
National Category
Neurosciences
Identifiers
URN: urn:nbn:se:liu:diva-162062DOI: 10.1523/JNEUROSCI.1214-19.2019ISI: 000493787000015PubMedID: 31530644OAI: oai:DiVA.org:liu-162062DiVA, id: diva2:1371354
Note

Funding Agencies|National Institutes of Health/National Institute of Neurological Disorders and StrokeUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Neurological Disorders & Stroke (NINDS) [NS095157]; UCLA Faculty Research Grant; National Institutes of Health/National Heart, Lung, and Blood InstituteUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Heart Lung & Blood Institute (NHLBI) [1R01HL134346]; David Vickter Foundation; Toeffler Family & Simon-Strauss Foundation; National Institutes of HealthUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [R01CA196263]; Knut and Alice Wallenberg FoundationKnut & Alice Wallenberg Foundation

Available from: 2019-11-19 Created: 2019-11-19 Last updated: 2019-11-19

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