Striking while the iron is hot: Iron metabolism and ferroptosis in neurodegeneration

Free Radic Biol Med. 2019 Mar:133:221-233. doi: 10.1016/j.freeradbiomed.2018.09.033. Epub 2018 Sep 25.

Abstract

Perturbations in iron homeostasis and iron accumulation feature in several neurodegenerative disorders including Alzheimer's disease (AD), Parkinson's disease (PD) and Amyotrophic lateral sclerosis (ALS). Proteins such as α-synuclein, tau and amyloid precursor protein that are pathologically associated with neurodegeneration are involved in molecular crosstalk with iron homeostatic proteins. Quantitative susceptibility mapping, an MRI based non-invasive technique, offers proximal evaluations of iron load in regions of the brain and powerfully predicts cognitive decline. Further, small molecules that target elevated iron have shown promise against PD and AD in preclinical studies and clinical trials. Despite these strong links between altered iron homeostasis and neurodegeneration the molecular biology to describe the association between enhanced iron levels and neuron death, synaptic impairment and cognitive decline is ill defined. In this review we discuss the current understanding of brain iron homeostasis and how it may be perturbed under pathological conditions. Further, we explore the ramifications of a novel cell death pathway called ferroptosis that has provided a fresh impetus to the "metal hypothesis" of neurodegeneration. While lipid peroxidation plays a central role in the execution of this cell death modality the removal of iron through chelation or genetic modifications appears to extinguish the ferroptotic pathway. Conversely, tissues that harbour elevated iron may be predisposed to ferroptotic damage. These emerging findings are of relevance to neurodegeneration where ferroptotic signalling may offer new targets to mitigate cell death and dysfunction.

Keywords: Alzheimer's disease; Ferroptosis; Iron; Neurodegeneration; Parkinson's disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Alzheimer Disease / drug therapy
  • Alzheimer Disease / metabolism*
  • Alzheimer Disease / pathology
  • Amyotrophic Lateral Sclerosis / drug therapy
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology
  • Brain / drug effects
  • Brain / metabolism
  • Brain / pathology
  • Cognitive Dysfunction / drug therapy
  • Cognitive Dysfunction / metabolism
  • Cognitive Dysfunction / pathology
  • Ferroptosis / drug effects
  • Humans
  • Iron / metabolism*
  • Iron Chelating Agents / therapeutic use
  • Lipid Peroxidation / drug effects
  • Neuroprotective Agents / therapeutic use
  • Parkinson Disease / drug therapy
  • Parkinson Disease / metabolism*
  • Parkinson Disease / pathology
  • Small Molecule Libraries / therapeutic use

Substances

  • Iron Chelating Agents
  • Neuroprotective Agents
  • Small Molecule Libraries
  • Iron