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Amino Acids

, Volume 49, Issue 7, pp 1147–1157 | Cite as

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 SquitieriEmail author
  • Andrea IlariEmail author
Review Article

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.

Keywords

Huntington disease Energetic metabolism Glucose transport GLUT1 GLUT3 

Notes

Acknowledgements

We thank the Foundation “Lega Italiana Ricerca Huntington e malattie correlate” (http://www.lirh.it) for supporting research on HD (FS) and taking care of families.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethics statement

This research did not involve human participants and/or animals.

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Copyright information

© Springer-Verlag Wien 2017

Authors and Affiliations

  • Veronica Morea
    • 1
  • Eris Bidollari
    • 2
    • 3
  • Gianni Colotti
    • 1
  • Annarita Fiorillo
    • 1
  • Jessica Rosati
    • 3
  • Lidia De Filippis
    • 3
  • Ferdinando Squitieri
    • 4
    Email author
  • Andrea Ilari
    • 1
    Email author
  1. 1.National Research Council of Italy (CNR), Institute of Molecular Biology and Pathology c/o Department of Biochemical SciencesSapienza University of RomeRomeItaly
  2. 2.Department of Biochemical SciencesSapienza University of RomeRomeItaly
  3. 3.IRCCS Casa Sollievo della Sofferenza HospitalSan Giovanni RotondoItaly
  4. 4.Huntington and Rare Diseases UnitIRCCS Casa Sollievo della Sofferenza Hospital c/o Mendel Institute of Human GeneticsRomeItaly

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