title: Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure?
abstract: Huntington's disease (HD) or Huntington's chorea is the most common inherited, dominantly transmitted, neurodegenerative disorder. It is caused by increased CAG repeats number in the gene coding for huntingtin (Htt) and characterized by motor, behaviour and psychiatric symptoms, ultimately leading to death. HD patients also exhibit alterations in glucose and energetic metabolism, which result in pronounced weight loss despite sustained calorie intake. Glucose metabolism decreases in the striatum of all the subjects with mutated Htt, but affects symptom presentation only when it drops below a specific threshold. Recent evidence points at defects in glucose uptake by the brain, and especially by neurons, as a relevant component of central glucose hypometabolism in HD patients. Here we review the main features of glucose metabolism and transport in the brain in physiological conditions and how these processes are impaired in HD, and discuss the potential ability of strategies aimed at increasing intracellular energy levels to counteract neurological and motor degeneration in HD patients.
authors.affiliation: National Research Council of Italy (CNR), Institute of Molecular Biology and Pathology c/o Department of Biochemical Sciences, Sapienza University of Rome, P.le A. Moro 5, 00185, Rome, Italy.
authors.forename: Veronica
authors.initials: V
authors.lastname: Morea
authors.affiliation: Department of Biochemical Sciences, Sapienza University of Rome, P.le A. Moro 5, 00185, Rome, Italy.|IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.
authors.forename: Eris
authors.initials: E
authors.lastname: Bidollari
authors.affiliation: National Research Council of Italy (CNR), Institute of Molecular Biology and Pathology c/o Department of Biochemical Sciences, Sapienza University of Rome, P.le A. Moro 5, 00185, Rome, Italy.
authors.forename: Gianni
authors.initials: G
authors.lastname: Colotti
authors.affiliation: National Research Council of Italy (CNR), Institute of Molecular Biology and Pathology c/o Department of Biochemical Sciences, Sapienza University of Rome, P.le A. Moro 5, 00185, Rome, Italy.
authors.forename: Annarita
authors.initials: A
authors.lastname: Fiorillo
authors.affiliation: IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.
authors.forename: Jessica
authors.initials: J
authors.lastname: Rosati
authors.affiliation: IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.
authors.forename: Lidia
authors.initials: L
authors.lastname: De Filippis
authors.affiliation: Huntington and Rare Diseases Unit, IRCCS Casa Sollievo della Sofferenza Hospital c/o Mendel Institute of Human Genetics, Viale Regina Margherita, 00198, Rome, Italy. f.squitieri@css-mendel.it.
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Squitieri
authors.affiliation: National Research Council of Italy (CNR), Institute of Molecular Biology and Pathology c/o Department of Biochemical Sciences, Sapienza University of Rome, P.le A. Moro 5, 00185, Rome, Italy. andrea.ilari@uniroma1.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ilari
mesh_terms: D001693:Biological Transport, Active / Q000235:genetics; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D004734:Energy Metabolism; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D000071058:Huntingtin Protein / Q000235:genetics / Q000378:metabolism; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D019680:Trinucleotide Repeat Expansion
chemical_list: C086055:HTT protein, human; D000071058:Huntingtin Protein; D005947:Glucose
keywords: Energetic metabolism; GLUT1; GLUT3; Glucose transport; Huntington disease