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Complement upregulation and activation on motor neurons and neuromuscular junction in the SOD1 G93A mouse model of familial amyotrophic lateral sclerosis

Heurich, Bianca, el Idrissi, Nawal Bahia, Donev, Rossen Mintchev, Petri, Susanne, Claus, Peter, Neal, James, Morgan, Bryan Paul ORCID: https://orcid.org/0000-0003-4075-7676 and Ramaglia, Valeria 2011. Complement upregulation and activation on motor neurons and neuromuscular junction in the SOD1 G93A mouse model of familial amyotrophic lateral sclerosis. Journal of Neuroimmunology 235 (1-2) , pp. 104-109. 10.1016/j.jneuroim.2011.03.011

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Abstract

Complement activation products are elevated in cerebrospinal fluid, spinal cord and motor cortex of patients with amyotrophic lateral sclerosis (ALS) but are untested in models. We determined complement expression and activation in the SOD1 G93A mouse model of familial ALS (fALS). At 126 days, C3 mRNA was upregulated in spinal cord and C3 protein accumulated in astrocytes and motor neurons. C3 activation products C3b/iC3b were localized exclusively on motor neurons. At the neuromuscular junction, deposits of C3b/iC3b and C1q were detected at day 47, before the appearance of clinical symptoms, and remained detectable at symptomatic stage (126 days). Our findings implicate complement in the denervation of the muscle endplate by day 47 and destruction of the neuromuscular junction and spinal neuron loss by day 126 in the SOD1 G93A mouse model of fALS.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Subjects: R Medicine > RC Internal medicine > RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry
Uncontrolled Keywords: amyotrophic lateral sclerosis, complement, motor neurons, neuromuscular junction
Publisher: Elsevier
ISSN: 0165-5728
Last Modified: 06 Dec 2022 09:52
URI: https://orca.cardiff.ac.uk/id/eprint/26025

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