title: Ketogenic diet in neuromuscular and neurodegenerative diseases.
abstract: An increasing number of data demonstrate the utility of ketogenic diets in a variety of metabolic diseases as obesity, metabolic syndrome, and diabetes. In regard to neurological disorders, ketogenic diet is recognized as an effective treatment for pharmacoresistant epilepsy but emerging data suggests that ketogenic diet could be also useful in amyotrophic lateral sclerosis, Alzheimer, Parkinson's disease, and some mitochondriopathies. Although these diseases have different pathogenesis and features, there are some common mechanisms that could explain the effects of ketogenic diets. These mechanisms are to provide an efficient source of energy for the treatment of certain types of neurodegenerative diseases characterized by focal brain hypometabolism; to decrease the oxidative damage associated with various kinds of metabolic stress; to increase the mitochondrial biogenesis pathways; and to take advantage of the capacity of ketones to bypass the defect in complex I activity implicated in some neurological diseases. These mechanisms will be discussed in this review.
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via Marzolo 3, 35031 Padova, Italy.
authors.forename: Antonio
authors.identifier: 0000-0003-0474-4229
authors.initials: A
authors.lastname: Paoli
authors.affiliation: Sport and Exercise Sciences Research Unit, University of Palermo, Via Eleonora Duse 2, 90146 Palermo, Italy.
authors.forename: Antonino
authors.identifier: 0000-0001-8334-6581
authors.initials: A
authors.lastname: Bianco
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via Marzolo 3, 35031 Padova, Italy.
authors.forename: Ernesto
authors.initials: E
authors.lastname: Damiani
authors.affiliation: Department of Biomedical Sciences, University of Padova, Via Marzolo 3, 35031 Padova, Italy.
authors.forename: Gerardo
authors.initials: G
authors.lastname: Bosco
mesh_terms: D020155:3-Hydroxybutyric Acid / Q000378:metabolism*; D000544:Alzheimer Disease / Q000178:diet therapy / Q000378:metabolism; D000690:Amyotrophic Lateral Sclerosis / Q000178:diet therapy / Q000378:metabolism; D001921:Brain / Q000378:metabolism; D055423:Diet, Ketogenic; D005947:Glucose / Q000378:metabolism*; D006008:Glycogen Storage Disease / Q000178:diet therapy / Q000378:metabolism; D006801:Humans; D028361:Mitochondrial Diseases / Q000178:diet therapy* / Q000378:metabolism; D010300:Parkinson Disease / Q000178:diet therapy / Q000378:metabolism
chemical_list: D005947:Glucose; D020155:3-Hydroxybutyric Acid
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