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Pathogenic mechanisms
A14 Fast axonal transport of mitochondria is altered in Huntington's disease
  1. L E Clemens1,
  2. A Grierson2,
  3. K De Vos3,
  4. O Riess1,
  5. H P Nguyen1
  1. 1Department of Medical Genetics, University of Tuebingen, Tuebingen, Germany
  2. 2Academic Neurology Unit, School of Medicine and Biomedical Sciences, University of Sheffield, Sheffield, UK
  3. 3MRC Centre for Neurodegeneration Research, Institute of Psychiatry, King's College London, London, UK


Background Expression of mutant huntingtin in mammalian neurons impairs both transport of vesicles and mitochondrial function. For this reason, it seems likely that fast axonal transport of mitochondria, which is crucial for neurite outgrowth and synaptic transmission, is affected as well.

Aim and method To investigate the influence of mutant huntingtin on fast axonal transport of mitochondria, we prepare primary neuronal cultures of cortex and striatum from mouse (HDKI mice) and rat (tgHD rats, BacHD rats) models of HD. Cells are transfected with EGFP and DsRed2Mito to track mitochondrial movements by time lapse video microscopy.

Result Analysis of the first recordings revealed an alteration in fast axonal transport, characterised by a significant increase in retrograde transport of mitochondria in transgenic HD rats compared with their wild-type littermates.

Conclusion Our study shows a shift in fast axonal transport of mitochondria towards more retrograde movements. Whether this is a common effect among HD animal models is currently under investigation.

  • fast axonal transport
  • huntingtin
  • mitochondria

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