A Commentary on Mitochondrial Dysfunction and Compromised DNA Repair in Neurodegeneration: The Emerging Role of FUS in ALS
Manohar Kodavati, Muralidhar L Hegde · 2024
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Description
Manohar Kodavati and Muralidhar Hegde comment on new evidence tying a familiar ALS protein to mitochondrial DNA damage, in a 2024 commentary. Mitochondrial failure runs through ALS, Alzheimer's, and Parkinson's diseases. Damage to the mitochondria's own small genome, and the repair of it, has emerged as a key part of that story.
The commentary discusses the authors' findings on FUS, an RNA and DNA binding protein mutated in some ALS cases. Healthy FUS partners with a mitochondrial ligase to keep mitochondrial DNA intact. Mutant FUS cells carried more mitochondrial DNA damage, directly shown. Restoring repair by expressing a human ligase in mutant models recovered mitochondrial function. The authors read DNA repair as a therapeutic lever for neurodegeneration. Their focus is FUS-linked ALS, but they frame the lesson for the wider family of diseases.
Catalog record
| CREATOR | Manohar Kodavati, Muralidhar L Hegde |
|---|---|
| YEAR | 2024 |
| COLLECTION | PAPERS |
| FORMAT | TXT |
| FILE SIZE | 13 KB |
| VERSION | 1.0 |
| LICENSE | CC BY 4.0 |
| RIGHTS | CC BY |
| SOURCE | Europe PMC |