Impaired mitochondrial energy production and ABC transporter function-A crucial interconnection in dementing proteopathies of the brain

Mech Ageing Dev. 2013 Oct;134(10):506-15. doi: 10.1016/j.mad.2013.08.007. Epub 2013 Sep 3.

Abstract

Ageing is the main risk factor for the development of dementing neurodegenerative diseases (NDs) and it is accompanied by the accumulation of variations in mitochondrial DNA. The resulting tissue-specific alterations in ATP production and availability cause deteriorations of cerebral clearance mechanisms that are important for the removal of toxic peptides and its aggregates. ABC transporters were shown to be the most important exporter superfamily for toxic peptides, e.g. β-amyloid and α-synuclein. Their activity is highly dependent on the availability of ATP and forms a directed energy-exporter network, linking decreased mitochondrial function with highly impaired ABC transporter activity and disease progression. In this paper, we describe a network based on interactions between ageing, energy metabolism, regeneration, accumulation of toxic peptides and the development of proteopathies of the brain with a focus on Alzheimer's disease (AD). Additionally, we provide new experimental evidence for interactions within this network in regenerative processes in AD.

Keywords: ABC transporters; ABCA1; ABCA7; ABCB1; ABCC1; Ageing; Alzheimer's disease; Aqp4; Dementia; Energy metabolism; Mitochondria; Neurodegeneration; Parkinson's disease.

Publication types

  • Review

MeSH terms

  • ATP-Binding Cassette Transporters / genetics
  • ATP-Binding Cassette Transporters / metabolism*
  • Aging / genetics
  • Aging / metabolism*
  • Aging / pathology
  • Alzheimer Disease / genetics
  • Alzheimer Disease / metabolism*
  • Alzheimer Disease / pathology
  • Amyloid beta-Peptides / genetics
  • Amyloid beta-Peptides / metabolism
  • Animals
  • Brain / metabolism*
  • Brain / pathology
  • Energy Metabolism / genetics
  • Humans
  • Mice
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism

Substances

  • ATP-Binding Cassette Transporters
  • Amyloid beta-Peptides
  • alpha-Synuclein