Cardiff University | Prifysgol Caerdydd ORCA
Online Research @ Cardiff 
WelshClear Cookie - decide language by browser settings

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 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

Full text not available from this repository.

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: 08 Jan 2018 21:33
URI: http://orca-mwe.cf.ac.uk/id/eprint/26025

Citation Data

Cited 24 times in Google Scholar. View in Google Scholar

Cited 28 times in Scopus. View in Scopus. Powered By Scopus® Data

Actions (repository staff only)

Edit Item Edit Item