Article Text

C8 Metabolic properties of hypothalamic primary neuron cultures from bachd rats
  1. Benedikt Fabry1,
  2. Laura Emily Clemens1,
  3. Stefanie Flunkert1,
  4. Hoa Huu Phuc Nguyen2,
  5. Robert Wronski1,
  6. Birgit Hutter-Paier1
  1. 1QPS Austria GmbH, Grambach, Austria
  2. 2Institute of Medical Genetics and Applied Genomics, Tübingen, Germany


Background The BACHD rat overexpresses a bacterial artificial chromosome (BAC) with the full length human mutant huntingtin (mHTT) with 97 alternating CAA/CAG repeats, which are the sole cause of disease onset. BACHD rats display mHTT aggregates and nuclear accumulation of mHTT throughout the brain and develop behavioural and neuropathological- and metabolic abnormalities, growth deficits, mitochondrial dysfunction as well as obesity and a lack of body weight loss that are not typical for human HD.

Aims The aim of this study was to investigate different metabolic properties and possible mitochondrial deficits in primary neurons of BACHD rats.

Methods BACHD rat pups were dissected at embryonic day 19 and primary neurons of the hypothalamus, striatum and cortex were cultivated and used for following assays:

  • MTT- and LDH-assays and IGF-1 levels for general health and growth.

  • MitoTracker measurements and JC-1-assay to assess the mitochondrial membrane potential

  • YO-PRO-1-Assay for the rate of mitochondrial apoptosis

Results Hypothalamic neuron cultures had a lower overall metabolic activity than primary neurons of WT controls. These cells also showed an increased survival rate compared to control cells. The same effects were observed in the striatum, whereas cells from the cortex showed no significant reduction of the metabolic activity, but a decreased survival rate. Since the experiments are still onging at the date of the abstract submission no further results can be shown.

Conclusion Our preliminary data and upcoming results will establish BACHD primary neurons as a valuable tool for the investigation of metabolic dysfunction in HD.

  • Primary neurons
  • Metabolism
  • Mitochondria

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