Characterising the phenotype of an isogenic, human iPSC-derived Huntington’s disease model by MEA

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Currently, no effective cure or treatment for Huntington’s disease exists. To accelerate Huntington’s disease research and drug discovery, researchers need access to physiologically relevant human disease models that recapitulate patient phenotypes, in combination with more advanced methods to understand the underlying mechanisms of the disease.

In this App Note you will learn:

  • How Charles River Laboratories characterised the phenotype of a human iPSC-derived Huntington’s disease model from over 38 days in vitro with high-density microelectrode arrays from MaxWell Biosystems.
  • How the disease model demonstrates physiologically relevant phenotypes, including delayed neuronal network formation, decreased axonal branching and decreased spontaneous activity compared to the isogenic control.
  • How the Huntington’s disease model from can be used with a genetically matched control, forming an isogenic system that allows scientists to attribute experimental outcomes directly to the disease-causing mutation.

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