What is the role of adenosine tone and adenosine receptors in huntington’s disease?

David Blum, En Chiang Chern, Maria Rosaria Domenici, Luc Buée, Ching Yeh Lin, Sergi Ferré, Patrizia Popoli

Research output: Contribution to journalArticle


Huntington’s disease (HD) is a devastating hereditary neurodegenerative disorder caused by a CAG mutation within the IT15 gene encoding huntingtin protein. Even though mutant and normal huntingtin are ubiquitously expressed, the degenerative processes primarily occur within the striatum and particularly hit the striatopallidal neurons, particularly enriched with adenosine A 2A receptors (A 2A R), suggesting that the latter might play a role in HD. In agreement, variants in the ADORA2A gene influence the age at onset in HD, and A 2A R dynamics is largely altered by mutated huntingtin. More generally, adenosine tone and adenosine receptors are involved in a number of processes critical for neuronal function and homeostasis, such as the modulation of synaptic activity and excitotoxicity, the control of neurotrophin levels and functions, and the regulation of protein degradation mechanisms. In the present review, we critically reviewed the current knowledge involving alterations of adenosine tone and adenosine receptors in HD and discussed whether they represent suitable therapeutic targets.

Original languageEnglish
Pages (from-to)281-308
Number of pages28
Publication statusPublished - Jan 1 2018


  • A heteromers
  • Adenosine receptors
  • Huntington’s disease
  • Neurotransmission

ASJC Scopus subject areas

  • Medicine(all)

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