Characterization of Striatal Neurons Derived from >140 CAG iPSCs for Huntington’s Disease Modeling

The poster Characterization of Striatal Neurons Derived from >140 CAG iPSCs for Huntington’s Disease Modeling presents Axol Bioscience’s development of a physiologically relevant HD model using patient-derived iPSCs with somatic CAG repeat instability.

Key highlights include:

• Genetic Characterization:
  • Initial CAG repeat count: 144
  • Expansion rate: +1 CAG every 5 passages
  • Loss of CAA interruption and presence of SNPs/INDELs linked to disease progression
• Striatal Neuron Differentiation:
  • HD and control lines differentiated using Axol’s proprietary media and protocols
  • HD neurons show smaller cell bodies and increased neurite branching
• Marker Expression:
  • Both HD and control neurons express DARPP32, CTIP2, GABA, and MAP2
  • Indicative of mature striatal neuron phenotype
• Electrophysiological Activity:
  • MEA analysis shows HD neurons are more active than controls at Day 15 and Day 25
  • Increased spike frequency and amplitude; broader electrode activation
• Somatic Instability:
  • Longitudinal analysis confirms progressive CAG expansion
  • Supports use in modeling disease onset and progression

This model supports target validation, phenotypic screening, and mechanistic studies in Huntington’s disease.

Featured Axol Products:

• HD iPSCs (>140 CAG)
• axoCells™ NSCs (HD background)
• axoCells™ Striatal Neuron Differentiation Media