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Self-inactivating AAV-CRISPR at different ages enables sustained amelioration of Huntington's disease deficits in BAC226Q mice - PubMed

3 hours ago
  • #Gene Therapy
  • #CRISPR-Cas9
  • #Huntington's Disease
  • Self-inactivating AAV-CRISPR system targets mutant HTT (mHTT) gene in Huntington's disease (HD).
  • CRISPR-Cas9 gene editing successfully eliminates mHTT gene, reducing protein and aggregation in BAC226Q mice.
  • Significant long-term improvements in neuropathology, motor deficits, weight loss, and lifespan observed.
  • Effective at various disease stages—before, at, and after symptom onset.
  • Self-inactivating system enhances safety by preventing prolonged Cas9 activity and off-target effects.
  • Proof-of-concept supports gene editing as a viable therapeutic approach for HD.