Iron dysregulation in Huntington's disease.
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.
Department of Medical Genetics, Centre for Molecular Medicine & Therapeutics, University of British Columbia and Children's and Women's Hospital, Vancouver, British Columbia, Canada.
Michelle
M
Muller
Blair R
BR
Leavitt
D000375:Aging / Q000378:metabolism; D000818:Animals; D001923:Brain Chemistry; D001927:Brain Diseases / Q000378:metabolism; D004195:Disease Models, Animal; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D007501:Iron / Q000378:metabolism*; D051379:Mice; D009154:Mutation / Q000235:genetics / Q000502:physiology; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism
C506812:Htt protein, mouse; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D007501:Iron
Huntington's disease; huntingtin; iron; magnetic resonance imaging; microglia; neurodegeneration