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Iron dysregulation in Huntington's disease

Michelle Muller1, Blair R Leavitt

  • 1Department of Medical Genetics, Centre for Molecular Medicine & Therapeutics, University of British Columbia and Children's and Women's Hospital, Vancouver, British Columbia, Canada.

Journal of Neurochemistry
|April 11, 2014
PubMed

Insights

Huntington's disease (HD) may involve altered brain iron, but evidence is unclear. Further research is needed to determine if iron accumulation contributes to HD neuropathogenesis or is a secondary effect.

Area of Science:

  • Neuroscience
  • Neurodegenerative Diseases
  • Iron Metabolism

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder.
  • Altered brain iron homeostasis is implicated in HD neuropathogenesis.
  • Previous studies suggest iron accumulation in specific brain regions affected by HD.

Purpose of the Study:

  • To investigate the role of brain iron in Huntington's disease.
  • To clarify whether elevated iron levels are a cause or consequence of HD.

Main Methods:

  • Review of post-mortem tissue studies.
  • Analysis of magnetic resonance imaging (MRI) findings.
  • Evaluation of biochemical assays measuring iron species.

Main Results:

  • MRI studies consistently report altered signals in neurodegenerative areas, interpreted as changes in brain iron.
  • Biochemical studies on human samples, mouse tissue, and in vitro have yielded conflicting data regarding iron levels.
  • The precise role and presence of elevated brain iron in HD remain uncertain.

Conclusions:

  • The association between altered brain iron and Huntington's disease requires further investigation.
  • Current evidence is equivocal regarding iron's contribution to HD pathogenesis.
  • It remains unclear if increased brain iron is a primary factor or a secondary effect in HD.

Keywords:
Huntington's diseasehuntingtinironmagnetic resonance imagingmicroglianeurodegeneration

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