The Amino Acid Cysteine May Hold the Key to Stopping Huntington's Disease

Volume 13    |   Issue 47

If you or a loved one has experienced Huntington's disease, you know how devastating this fatal neurodegenerative disorder is. However, researchers at Johns Hopkins have recently made a connection between this disease and a particular amino acid. While Huntington's disease is pretty rare, these findings may have implications for other neurodegenerative conditions and diseases that involve deficiencies of this amino acid and oxidative stress, including Alzheimer's, arthritis, cardiovascular disease, and cancer.

According to the researchers' findings, which they published in the Proceedings of the National Academy of Sciences, oxidative stress damages nerve cells in people with Huntington's disease. This isn't particularly surprising — after all, oxidative stress damages a lot of cells in a lot of people, with or without Huntington's disease. But what the researchers were able to uncover is why these particular nerve cells endure an onslaught of oxidative stress that they simply can't keep up with.

This attack has to do with an amino acid called cysteine. For most of us, cysteine plays a major role in helping us deal with oxidative stress. But the researchers found that people with Huntington's disease have low levels of the protein that makes cysteine, called cystathionine gamma-lyase (CSE). Normally, when cysteine gets low, cells signal another protein, called activating transcription factor 4 (ATF4) to tell CSE to get to work. But in people with Huntington's disease, ATF4 never delivers the message. CSE doesn't make enough cysteine, and oxidative stress starts to take over.

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Interestingly, when the researchers gave cysteine-depleted Huntington's cells the antioxidant vitamin C, the cells were able to create enough CSE to make more cysteine. The vitamin C may have lifted the oxidative stress burden enough to allow ATF4 to deliver the message. They also found that giving mice with Huntington's disease supplemental cysteine through their diets helped slow the progression of the disease. These are both promising findings for helping researchers eventually develop effective therapies for Huntington's disease and other diseases affected by depleted cysteine levels.

You can get antioxidants, including vitamin C, from your diet. But to really ensure you're protected with significant amounts, it's best to take a supplement. Few of us, including me, eat as well as we could every single day, so I like the peace of mind the additional protection of a supplement offers. Advanced Polyphenol Formula is one of my favorite ways to get extra antioxidants.

While antioxidants aren't currently sufficient to cure Huntington's disease, they are important. And this line of research is promising for people who have a genetic tendency and/or suffer from the disease.







Juan I. Sbodio, Solomon H. Snyder, Bindu D. Paul. Transcriptional control of amino acid homeostasis is disrupted in Huntington’s disease. Proceedings of the National Academy of Sciences, 2016; 201608264 DOI: 10.1073/pnas.1608264113.

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