%20is%20a%20rare%20genetic%20disorder%20that%20causes%20early%20aging%20and%20progressive%20brain%20degeneration%20due%20to%20problems%20in%20DNA%20repair%20genes%20(CSA%20or%20CSB).%20In%20mice%20with%20the%20CSB%20mutatio...){kind=link}
A High-Fat Diet and Increasing NAD+ Rescues Premature Aging in Cockayne Syndrome: Preclinical Findings
Synopsis
Cockayne syndrome (CS) is a rare genetic disorder that causes early aging and progressive brain degeneration due to problems in DNA repair genes (CSA or CSB). In mice with the CSB mutation, researchers found that diet strongly affects disease progression. A high-fat diet improved many symptoms—including metabolism, gene activity, and behavior—by restoring key cellular processes. The study showed that faulty DNA repair overactivates the PARP enzyme, which lowers NAD+ levels and reduces SIRT1 activity, causing mitochondrial problems and faster aging. Boosting β-hydroxybutyrate with a high-fat diet, or directly increasing NAD+ or blocking PARP, reactivated SIRT1 and improved cell health. These results suggest that nutritional or metabolic strategies targeting NAD+ and SIRT1 could help treat neurological and aging-related symptoms in Cockayne syndrome and possibly other neurodegenerative diseases.
Journal
Cell Metabolism