Metabolic Therapy with Deanna Protocol Supplementation Delays Disease Progression and Extends Survival in Amyotrophic Lateral Sclerosis (ALS) Mouse Model

• DOI: 10.1371/journal.pone.0103526
• Publication Date: 2014-07-25T18:04:34Z
• Authors: Csilla Ari; Angela M. Poff; Heather E. Held; Carol S. Landon; Craig R. Goldhagen; Nicholas Mavromates; Dominic P. D’Agos...
• Keywords: Male; Superoxide Dismutase; Ubiquinone; Science; Q; Amyotrophic Lateral Sclerosis; R; Arginine; 3. Good health; Mice; 03 medical and health sciences; Superoxide Dismutase-1; 0302 clinical medicine; Dietary Supplements; Medicine; Animals; Ketoglutaric Acids; Caprylates; gamma-Aminobutyric Acid; Research Article

Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, is a neurodegenerative disorder of motor neurons causing progressive muscle weakness, paralysis, and eventual death from respiratory failure. There currently no cure or effective treatment for ALS. Besides neuron degeneration, ALS associated with impaired energy metabolism, which pathophysiologically linked to mitochondrial dysfunction glutamate excitotoxicity. The Deanna Protocol (DP) metabolic therapy that has been reported alleviate symptoms in patients In this study we hypothesized alternative fuels the form TCA cycle intermediates, specifically arginine-alpha-ketoglutarate (AAKG), main ingredient DP, ketogenic diet (KD), would increase function survival mouse model (SOD1-G93A). mice were fed standard rodent (SD), KD, either diets containing primary ingredients DP consisting AAKG, gamma-aminobutyric acid, Coenzyme Q10, medium chain triglyceride high caprylic triglyceride. Assessment ALS-like pathology was performed using pre-defined criteria neurological score, accelerated rotarod test, paw grip endurance strength test. Blood glucose, blood beta-hydroxybutyrate, body weight monitored. SD+DP-fed exhibited improved score age 116 136 days compared control mice. KD-fed better performance on all tests at 15 16 weeks controls. SD+DP KD+DP therapies significantly extended time SOD1-G93A by 7.5% (p = 0.001) 4.2% 0.006), respectively. Sixty-three percent 72.7% group lived past 125 days, while only 9% animals survived point. Targeting metabolism produces therapeutic effect may prolong quality life patients.