Mitochondrial dysfunction in Wilson disease: a systematic review and meta–analysis across human and animal models
Raya Amin, Valentina Medici, Erik D. Fausak, Keren Sierra, Ieleen Li, Joshua Gong, Cecilia Giulivi · 2025
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Description
Raya Amin and co-authors pool evidence on cell power units in Wilson disease in a 2025 review. The genetic disease traps copper in the body, above all in the liver. Mitochondria, the cell's power plants, seem especially open to copper harm.
Thirteen human and animal studies met their rules. Copper inside liver mitochondria ran far above normal, and their inner structure looked abnormal. Stress markers rose, while energy output and key respiratory steps fell, more so in older or symptomatic cases. DNA copies inside mitochondria dropped too. Some flaws appeared before symptoms, hinting that power unit harm is an early driver of the disease.
Catalog record
| CREATOR | Raya Amin, Valentina Medici, Erik D. Fausak, Keren Sierra, Ieleen Li, Joshua Gong, Cecilia Giulivi |
|---|---|
| YEAR | 2025 |
| COLLECTION | PAPERS |
| FORMAT | TXT |
| FILE SIZE | 61 KB |
| VERSION | 1.0 |
| LICENSE | CC BY 4.0 |
| RIGHTS | CC BY |
| SOURCE | Europe PMC |