Mitochondrial Bioenergy in Neurodegenerative Disease: Huntington and Parkinson

Int J Mol Sci. 2023 Apr 13;24(8):7221. doi: 10.3390/ijms24087221.

Abstract

Strong evidence suggests a correlation between degeneration and mitochondrial deficiency. Typical cases of degeneration can be observed in physiological phenomena (i.e., ageing) as well as in neurological neurodegenerative diseases and cancer. All these pathologies have the dyshomeostasis of mitochondrial bioenergy as a common denominator. Neurodegenerative diseases show bioenergetic imbalances in their pathogenesis or progression. Huntington's chorea and Parkinson's disease are both neurodegenerative diseases, but while Huntington's disease is genetic and progressive with early manifestation and severe penetrance, Parkinson's disease is a pathology with multifactorial aspects. Indeed, there are different types of Parkinson/Parkinsonism. Many forms are early-onset diseases linked to gene mutations, while others could be idiopathic, appear in young adults, or be post-injury senescence conditions. Although Huntington's is defined as a hyperkinetic disorder, Parkinson's is a hypokinetic disorder. However, they both share a lot of similarities, such as neuronal excitability, the loss of striatal function, psychiatric comorbidity, etc. In this review, we will describe the start and development of both diseases in relation to mitochondrial dysfunction. These dysfunctions act on energy metabolism and reduce the vitality of neurons in many different brain areas.

Keywords: Huntington’s disease; Parkinson’s disease; basal ganglia; calcium; energy metabolism; mitochondria; movement disorders; synaptic plasticity.

Publication types

  • Review

MeSH terms

  • Brain / metabolism
  • Humans
  • Huntington Disease* / metabolism
  • Mitochondria / metabolism
  • Neurodegenerative Diseases* / metabolism
  • Parkinson Disease* / metabolism

Grants and funding

This work was partially supported by Italian Ministry of Health “Ricerca Finalizzata” grant RF-2019-12370182 to P.B. The funding source had no involvement in the design or writing of the report and in the decision to submit the article for publication.