Journal article
Modelling biochemical features of mitochondrial neuropathology
MJ Bird, DR Thorburn, AE Frazier
Biochimica Et Biophysica Acta General Subjects | ELSEVIER SCIENCE BV | Published : 2014
Abstract
Background The neuropathology of mitochondrial disease is well characterised. However, pathophysiological mechanisms at the level of biochemistry and cell biology are less clear. Progress in this area has been hampered by the limited accessibility of neurologically relevant material for analysis. Scope of review Here we discuss the recent development of a variety of model systems that have greatly extended our capacity to understand the biochemical features associated with mitochondrial neuropathology. These include animal and cell based models, with mutations in both nuclear and mitochondrial DNA encoded genes, which aim to recapitulate the neuropathology and cellular biochemistry of mitoch..
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Funding Acknowledgements
The authors' research was supported by grants from the Australian National Health and Medical Research Council (NHMRC), the Australian Mitochondrial Disease Foundation, an NHMRC Principal Research Fellowship to David Thorburn, an NHMRC Career Development Award to Ann Frazier, an Australian Postgraduate Award to Matthew Bird and the Victorian Government's Operational Infrastructure Support Program. We thank Alysha Eibl and Leila Varghese for helpful comments on the manuscript.