Journal article

Neurocardiac dysregulation and neurogenic arrhythmias in a transgenic mouse model of Huntington's disease

Helen Kiriazis, Nicole L Jennings, Pamela Davern, Gavin Lambert, Yidan Su, Terence Pang, Xin Du, Luisa La Greca, Geoffrey A Head, Anthony J Hannan, Xiao-Jun Du



Huntington's disease (HD) is a heritable neurodegenerative disorder, with heart disease implicated as one major cause of death. While the responsible mechanism remains unknown, autonomic nervous system (ANS) dysfunction may play a role. We studied the cardiac phenotype in R6/1 transgenic mice at early (3 months old) and advanced (7 months old) stages of HD. While exhibiting a modest reduction in cardiomyocyte diameter, R6/1 mice had preserved baseline cardiac function. Conscious ECG telemetry revealed the absence of 24-h variation of heart rate (HR), and higher HR levels than wild-type littermates in young but not older R6/1 mice. Older R6/1 mice had increased plasma level of noradrenaline (..

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Awarded by National Heart Foundation of Australia

Funding Acknowledgements

This work was supported by the National Heart Foundation of Australia (grant number G10M5126). A.J.H. was Future Fellow of the Australian Research Council; G. L., G. A. H. and X.J.D. were fellows of the National Health and Medical Research Council of Australia; X. D. was a recipient of a Melbourne University Research Scholarship. This study was supported in part by the Victorian Government's Operational Infrastructure Support Program.