Contribution to a book

DZNE-2022-01528

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png From Physiological Properties to Selective Vulnerability of Motor Units in Amyotrophic Lateral Sclerosis

O'Donovan, M. J. (Editor) ; Falgairolle, M. (Editor) ; Bączyk, M. ; Manuel, M. ; Roselli, F.DZNE* ; Zytnicki, D.

2022
Springer International Publishing Cham
ISBN: 978-3-031-07166-9 (print), 978-3-031-07167-6 (electronic)

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Please use a persistent id in citations: doi:10.1007/978-3-031-07167-6_15

Abstract: Spinal alpha-motoneurons are classified in several types depending on the contractile properties of the innervated muscle fibers. This diversity is further displayed in different levels of vulnerability of distinct motor units to neurodegenerative diseases such as Amyotrophic Lateral Sclerosis (ALS). We summarize recent data suggesting that, contrary to the excitotoxicity hypothesis, the most vulnerable motor units are hypoexcitable and experience a reduction in their firing prior to symptoms onset in ALS. We suggest that a dysregulation of activity-dependent transcriptional programs in these motoneurons alter crucial cellular functions such as mitochondrial biogenesis, autophagy, axonal sprouting capability and re-innervation of neuromuscular junctions.

Keyword(s): Amyotrophic Lateral Sclerosis (MeSH) ; Humans (MeSH) ; Motor Neurons (MeSH) ; Muscle Contraction (MeSH)

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Contributing Institute(s):

1. Metabolic Changes (AG Roselli)

Research Program(s):

1. 352 - Disease Mechanisms (POF4-352) (POF4-352)

Appears in the scientific report 2022

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