Genetics of Parkinson's disease and related disorders

J Med Genet. 2018 Feb;55(2):73-80. doi: 10.1136/jmedgenet-2017-105047. Epub 2017 Nov 18.

Abstract

Parkinson's disease (PD) is a complex and heterogeneous neurological condition characterised mainly by bradykinesia, resting tremor, rigidity and postural instability, symptoms that together comprise the parkinsonian syndrome. Non-motor symptoms preceding and following clinical onset are also helpful diagnostic markers revealing a widespread and progressive pathology. Many other neurological conditions also include parkinsonism as primary or secondary symptom, confounding their diagnosis and treatment. Although overall disease course and end-stage pathological examination single out these conditions, the significant overlaps suggest that they are part of a continuous disease spectrum. Recent genetic discoveries support this idea because mutations in a few genes (α-synuclein, LRRK2, tau) can cause partially overlapping pathologies. Additionally, mutations in causative genes and environmental toxins identify protein homeostasis and the mitochondria as key mediators of degeneration of dopaminergic circuits in the basal ganglia. The evolving mechanistic insight into the pathophysiology of PD and related conditions will contribute to the development of targeted and effective symptomatic treatments into disease-modifying therapies that will reduce the burden of these dreadful conditions.

Keywords: basal ganglia; disease mechanisms; genetics; parkinsonism; parkinson’s disease.

Publication types

  • Review

MeSH terms

  • Dopamine / metabolism
  • Genes, Dominant
  • Humans
  • Leucine-Rich Repeat Serine-Threonine Protein Kinase-2 / genetics*
  • Mitochondria / metabolism
  • Mitochondria / pathology
  • Mutation*
  • Neurons / metabolism
  • Neurons / pathology
  • Parkinson Disease / etiology
  • Parkinson Disease / genetics*
  • Parkinsonian Disorders / chemically induced
  • Parkinsonian Disorders / etiology*
  • Parkinsonian Disorders / pathology
  • alpha-Synuclein / genetics
  • tau Proteins / genetics
  • tau Proteins / metabolism

Substances

  • alpha-Synuclein
  • tau Proteins
  • LRRK2 protein, human
  • Leucine-Rich Repeat Serine-Threonine Protein Kinase-2
  • Dopamine