Loss of C9orf72 impacts the peripheral neuromuscular system via immune dysregulation and accelerates the progression of amyotrophic lateral sclerosis in SOD-1 mutant mice

Abstract Background Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder where neuromuscular health is central to disease progression. The degeneration of motor neurons (MNs) leads to muscle weakness and paralysis, underscoring the critical importance of neuromuscular junc...

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Detalhes bibliográficos

Principais autores:	Francesca Sironi, Massimo Tortarolo, Sabrina Mazzucchi, Laura Camporeale, Mattia Freschi, Eliana Arcadipane, Giulia De Giovanetti, Mami Kurosaki, Mineko Terao, Paola Parlanti, Paola Podini, Francesco Gentile, Valentina Bonetto, Valentina Cappello, Nilo Riva, Angelo Quattrini, Caterina Bendotti
Formato:	Artigo
Idioma:	Inglês
Publicado em:	BMC 2025-11-01
coleção:	Journal of Neuroinflammation
Assuntos:	C9orf72 Amyotrophic lateral sclerosis Peripheral nervous system SOD1G93A mice Immune system
Acesso em linha:	https://doi.org/10.1186/s12974-025-03597-y
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