Journal article

Dysregulation of the complement cascade in the hSOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis

John D Lee, Nur A Kamaruzaman, Jenny NT Fung, Stephen M Taylor, Bradley J Turner, Julie D Atkin, Trent M Woodruff, Peter G Noakes



BACKGROUND: Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of hSOD1(G93A) mice during defined disease stages. METHODS: hSOD1(G93A) and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting ..

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Awarded by NHMRC

Funding Acknowledgements

The authors would like to thank Maryam Shayegh and Mary White for their technical support and David Simmons for assistance in in situ hybridisation. JDL holds an APA scholarship from the Australian government. The work was funded by project grant APP1004455 from NHMRC to PGN and TMW, and by a MNDRIA project grant to PGN, SMT and TMW. BJT is supported by grants APP1008910 from NHMRC and MNDRIA, and JDA by grants APP1006141 and APP1030513from the NHMRC and grants from MNDRIA.