1. Department of Medical Genetics, Centre for Molecular Medicine & Therapeutics, University of British Columbia and Children's and Women's Hospital, , Vancouver, British Columbia, Canada;2. Division of Neurology, Department of Medicine, University of British Columbia Hospital, , Vancouver, British Columbia, Canada;3. Brain Research Centre, University of British Columbia, , Vancouver, British Columbia, Canada
Abstract:
Huntington's disease (HD) is one of many neurodegenerative diseases with reported alterations in brain iron homeostasis that may contribute to neuropathogenesis. Iron accumulation in the specific brain areas of neurodegeneration in HD has been proposed based on observations in post‐mortem tissue and magnetic resonance imaging studies. Altered magnetic resonance imaging signal within specific brain regions undergoing neurodegeneration has been consistently reported and interpreted as altered levels of brain iron. Biochemical studies using various techniques to measure iron species in human samples, mouse tissue, or in vitro has generated equivocal data to support such an association. Whether elevated brain iron occurs in HD, plays a significant contributing role in HD pathogenesis, or is a secondary effect remains currently unclear.
