Journal article
Neuronal and astrocyte dysfunction diverges from embryonic fibroblasts in the Ndufs4fky/fky mouse
MJ Bird, XW Wijeyeratne, JC Komen, A Laskowski, MT Ryan, DR Thorburn, AE Frazier
Bioscience Reports | Published : 2014
DOI: 10.1042/BSR20140151
Abstract
Synopsis Mitochondrial dysfunction causes a range of early-onset neurological diseases and contributes to neurodegenerative conditions. Themechanisms of neurological damage however are poorly understood, as accessing relevant tissue from patients is difficult, and appropriate models are limited. Hence, we assessed mitochondrial function in neurologically relevant primary cell lines from a CI (complex I) deficient Ndufs4 KO (knockout) mouse (Ndufs4fky/fky) modelling aspects of the mitochondrial disease LS (Leigh syndrome), as well as MEFs (mouse embryonic fibroblasts). Although CI structure and function were compromised in all Ndufs4fky/fky cell types, the mitochondrial membrane potential was..
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Awarded by National Health and Medical Research Council
Funding Acknowledgements
The authors' research was supported by grants from the Australian National Health and Medical Research Council (NHMRC) [grant number APP102222] and the Muscular Dystrophy Association (MDA) [grant number MDA113072], an NHMRC Principal Research Fellowship to David Thorburn [grant number 1022896], an NHMRC Career Development Award to Ann Frazier [grant number 541920], and an Australian Postgraduate Award to Matthew Bird [grant number APA2011]. Additional support was provided by the Australian Mitochondrial Disease Foundation and the Victorian Government's Operational Infrastructure Support Programme.