Huntington’s disease and selenoproteins

L-selenomethionine is metabolized by cystathionine gamma-lysase. Dysregulation of cystathionine gamma-lyase would dysregulate selenoprotein synthesis from L-selenomethionine, which is the food form of selenium. In the N171-82Q Huntington’s disease mouse model various selenoprotein levels are changed compared to controls. Changed levels of selenoproteins in the N171-82Q Huntington’s disease mouse model compared to controls could be due to dysregulation of cystathionine gamma-lyase.

Supplemental Se-methylselenocysteine could be useful in the treatment of Huntington’s disease as metabolism of Se-methylselenocysteine does not depend on cystathionine gamma-lyase. Se-methylselenocysteine is metabolized by kynurenine aminotransferase 1. Supplementation of Se-methylselenocysteine taken alone would be far from a completely effective treatment for Huntington’s disease as decreases in cystathionine gamma-lyase would dysregulate various pathways downstream from the transsulfuration pathway. Se-methylselenocysteine could be of assistance in the treatment of cognitive symptoms of Huntington’s disease. Se-methylselenocysteine taken daily in two divided dosages is more effective than Se-methylselenocysteine taken once daily.

2 thoughts on “Huntington’s disease and selenoproteins

  1. Could you translate this very important writings in practical terms?
    Supplemental Se-methylselenocysteine ACOULD BE USEFUL in the treatment of Huntington’s disease as metabolism of Se-methylselenocysteine does not depend on cystathionine gamma-lyase. Se-methylselenocysteine is metabolized by kynurenine aminotransferase 1. Se-methylselenocysteine WOULD NOT BE a completely effective treatment for Huntington’s disease as…….
    YES OR NO to prescribe Se-methylselenocysteine [would brazil nuts do as well?]

    1. Se-methylselenocysteine taken daily in two divided dosages could be useful in the treatment of cognitive symptoms of Huntington’s disease, however, as of yet there have been no clinical trials of Se-methylselenocysteine in the treatment of Huntington’s disease. Correct dosing of Se-methylselenocysteine is a key to successful treatment with Se-methylselenocysteine. Selenium can be quite toxic. One hundred micrograms of Se-methylselenocysteine taken twice a day is where treatment appropriately starts. Given Se-methylselenocysteine is taken only once dailly there is an end-of-dosage effect around late afternoon. Given Se-methylselenocysteine is taken as a stand-alone supplement no more than 200 micrograms of Se-methylselenocysteine is taken daily. Se-methylselenocysteine would be far from a completely effective treatment for Huntington’s disease.

      Brazil nuts would not work. Brazil nuts largely contain L-selenomethionine.

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