Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure?

Veronica Morea, Eris Bidollari, Gianni Colotti, Annarita Fiorillo, Jessica Rosati, Lidia De Filippis, Ferdinando Squitieri, Andrea Ilari

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish
Pages (from-to)1147-1157
Number of pages11
JournalAmino Acids
Volume49
Issue number7
DOIs
Publication statusPublished - Jul 1 2017

Keywords

  • Energetic metabolism
  • Glucose transport
  • GLUT1
  • GLUT3
  • Huntington disease

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry
  • Organic Chemistry

Fingerprint Dive into the research topics of 'Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure?'. Together they form a unique fingerprint.

Cite this