ALS is an adult-onset neurodegenerative disorder in which spinal and cortical motor neurons die causing relentlessly progressive weakness and wasting of skeletal muscles through the body.
"ALS is such a devastating disease for those individuals diagnosed with the disorder," said Giulio Maria Pasinetti, M.D., Ph.D., Professor of Psychiatry and Neuroscience, Director of the Neuroinflammation Research Center at The Mount Sinai School of Medicine and lead author of this study. "The findings assert the significance of certain high caloric dietary intake in the prevention of ALS. In view of any available therapeutic application for the disease, this new evidence might bring hope to those affected."
The cause of neuronal death in ALS is uncertain but study researchers say mitochondrial dysfunction plays an important role. Ketones promote mitochondrial energy production and membrane stabilization. Mitochondiral membrane dysfunction, loss of oxidative stress control, generation of excessive free radicals, neurofilament accumulation, and excitotoxicity are all implicated in the onset of ALS.
About the Study
Mount Sinai School of Medicine investigators used a mouse model to examine the affects of a ketogenic diet (KD) on the progression of ALS. ALS mice were fed a high caloric ketogenic diet (KD) and motor performance, longevity, and motor neuron counts were measured in treated and diseased mice. Because mitochondrial dysfunction plays a central role in neuronal cell death in ALS, the effect that the principal ketone body, D--3 hydroxybutyrate (DBH), has on mitochondrial ATP genera
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