Real-World Evidence Helps Open Accelerated Approval Pathway For Neurodegeneration Therapies

Clene’s ALS drug CNM-Au8 and UniQure’s gene therapy for Huntington’s disease emerged from US FDA discussions with accelerated approval plans using real-world evidence from compassionate use and natural history data sources.

Clene's ALS drug and uniQure's Huntington's disease gene therapy hope for a smooth road to accelerated approval despite the pathway's difficult history in neurodegenerative disease. (Shutterstock)
Key Takeaways
  • US FDA's agreements with plans to seek accelerated approval of Clene’s amyotrophic lateral sclerosis drug and uniQure’s Huntington’s disease gene therapy reflect growing pressure to expand the expedited pathways for neurodegenerative diseases.
  • Clene will use expanded access protocol data to support CNM-Au8’s effect on a surrogate endpoint measuring neurofilament light protein as part of a planned second quarter 2025 NDA submission.
  • UniQure expects to submit a BLA for AMT-130 based on an intermediate endpoint in Phase I/II trials compared to natural history controls.

Real-world evidence from expanded access programs and a natural history external control played important roles in recent US FDA decisions on the suitability of the accelerated approval pathway for

Neurodegenerative diseases have not fit neatly into the accelerated approval pathway, as illustrated by the controversies stemming from FDA approvals of Sarepta’s ALS...

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