Article: Nicotinamide Riboside Restores NAD+ and Improves Heart and Brain Function in Leigh Syndrome: Preclinical Findings

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Cardiovascular Health Neurological Health Orphan Disease Preclinical

Nicotinamide Riboside Restores NAD+ and Improves Heart and Brain Function in Leigh Syndrome: Preclinical Findings

Synopsis

Mice lacking the mitochondrial complex I subunit Ndufs4 develop Leigh syndrome (LS), marked by serious brain and heart problems. This study found these mice have slow, irregular heartbeats and poor heart relaxation linked to low NAD+/NADH levels and excessive acetylation of key proteins like the cardiac sodium channel NaV1.5. In the brain, neuron loss was tied to increased acetylation of p53 and immune activation. Supplementing with nicotinamide riboside (NR) restored NAD+ balance, reversed protein acetylation, improved heart function, and reduced brain cell death in both mice and human stem cell models. These results reveal how NAD+ deficiency harms heart and brain metabolism in LS and suggest NR as a potential treatment.

Journal

Clinical and Translational Medicine

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