Mitochondrial dysfunction in Wilson disease: a systematic review and meta–analysis across human and animal models
Across human and animal studies, hepatic mitochondria in WD exhibit copper accumulation, structural injury, impaired bioenergetics, oxidative stress, and mitochondrial genome loss. Mitochondrial dysfunction arises early and worsens with progression, highlighting it as a central pathogenic feature and therapeutic target.
Authors
- Erik Davis Fausak (ORCID: https://orcid.org/0000-0002-0510-0153)
- Joshua Gong (ORCID: https://orcid.org/0000-0001-5181-4862)
- Valentina Medici (ORCID: https://orcid.org/0000-0001-5438-284X)
- Karla A. Sierra
- Cecilia Giulivi (ORCID: https://orcid.org/0000-0003-1033-7435)
- I Anren Li (ORCID: https://orcid.org/0009-0001-5937-3878)
- Rajni Amin
Institutions
- NeuroDevelopment Center (US)
- University of California, Davis (US)
Publication Details
- Journal
- Frontiers in Molecular Biosciences
- Published
- 2025-12-17
- DOI
- https://doi.org/10.3389/fmolb.2025.1712573
- Primary Topic
- Trace Elements in Health
- Type
- article
- Field-Weighted Citation Impact
- 0.00