Sex-specific and age-related progression of auditory neurophysiological deficits in the Cln3 mouse model of Batten disease
These findings demonstrate sex- and age-dependent disruptions in central auditory processing in Cln3-/- mice and support auditory duration MMN as a sensitive, translational biomarker of brain dysfunction in CLN3 disease. This approach offers a functional, cross-species measure for tracking disease progression and evaluating therapeutic interventions in Batten disease.
Authors
- Alicia Solórzano (ORCID: https://orcid.org/0000-0003-0072-3463)
- Hyeyeon Chang (ORCID: https://orcid.org/0000-0001-6939-7913)
- Yanya Ding (ORCID: https://orcid.org/0000-0002-6343-926X)
- Edward G. Freedman (ORCID: https://orcid.org/0000-0001-8570-6325)
- Jingyu Feng (ORCID: https://orcid.org/0000-0003-3181-4544)
- John J. Foxe (ORCID: https://orcid.org/0000-0002-4300-3098)
- Kuan Hong Wang (ORCID: https://orcid.org/0000-0002-2249-5417)
- Viollandi Prifti (ORCID: https://orcid.org/0009-0006-3522-1374)
- Grace Rico
Institutions
- University of Rochester Medical Center (US)
Publication Details
- Journal
- Journal of Neurodevelopmental Disorders
- Published
- 2025-11-06
- DOI
- https://doi.org/10.1186/s11689-025-09652-2
- Primary Topic
- Lysosomal Storage Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- National Institutes of Health