Huntington’s Disease iPSCs-derived striatal neurons with 140 CAG repeats over a 100 day-culture

The Huntington’s Disease Striatal Neuron Poster from Axol Bioscience presents a physiologically relevant in vitro model using patient-derived iPSCs with >140 CAG repeats. This model enables long-term culture, functional characterization, and genetic profiling for HD research and therapeutic development.

Key highlights include:

• HD iPSC Line with >140 CAG Repeats: Shows somatic instability with repeat expansion every 5 passages; confirmed via Nanopore sequencing.
• Striatal Neuron Differentiation: Cells express MAP2, CTIP2, DARPP32 after 31 days; maintain phenotype for over 100 days.
• Functional Activity: HD striatal neurons show increased sodium spike frequency and amplitude compared to controls (MEA analysis).
• Morphological Differences: HD neurons form smaller clusters with more neurite branches than WT.
• Genetic Characterization: SNPs and INDELs identified in the mHTT allele; loss of CAA interruption linked to disease progression.
• Longitudinal Studies: CAG repeat expansion continues during maturation (0.13X/week); RNA-seq confirms stable marker expression.
• Antibody Optimization: HTT NEO P90 clones 1B12 and 11G2 tested for differential staining in HD vs. control neurons.
• Additional HD Lines: Axol has matured other patient-derived iPSC lines with 39–66 CAG repeats for comparative studies.
• Co-Culture Potential: Axol can generate cortical neurons, astrocytes, and microglia from the same HD genetic background for complex disease modeling.

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

Featured Axol Products:

• HD iPSCs – Patient-derived lines with varying CAG repeat lengths
• axoCells™ NSCs – Neural stem cells for striatal differentiation
• axoCells™ Striatal Neuron Differentiation Media  – Optimized for long-term culture and maturation
• axoCells™ Cortical Neurons, Astrocytes, Microglia – Derived from HD genetic background for co-culture models