Altered Manganese Homeostasis and Manganese Toxicity in a Huntington's Disease Striatal Cell Model Are Not Explained by Defects in the Iron Transport System

Abstract excerpt

Expansion of a polyglutamine tract in Huntingtin (Htt) leads to the degeneration of medium spiny neurons in Huntington's disease (HD). Furthermore, the HTT gene has been functionally linked to iron (Fe) metabolism, and HD patients show alterations in brain and peripheral Fe homeostasis. Recently, we discovered that expression of mutant HTT is associated with impaired manganese (Mn) uptake following overexposure...

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