The Role of Adenosine Tone and Adenosine Receptors in Huntington's Disease.

ABSTRACT: Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a mutation in the IT15 gene that encodes for the huntingtin protein. Mutated hungtingtin, although widely expressed in the brain, predominantly affects striato-pallidal neurons, particularly enriched with adenosine A_2A receptors (A_2AR), suggesting a possible involvement of adenosine and A_2AR is the pathogenesis of HD. In fact, polymorphic variation in the ADORA2A gene influences the age at onset in HD, and A_2AR dynamics is altered by mutated huntingtin. Basal levels of adenosine and adenosine receptors are involved in many processes critical for neuronal function and homeostasis, including modulation of synaptic activity and excitotoxicity, the control of neurot

SUBMITTER: Blum D 

PROVIDER: S-EPMC6049521 | biostudies-literature | 2018 Jun

REPOSITORIES: biostudies-literature