Huntington’s & Paleo

Huntington's & Paleo | The Paleo Diet

I just listened to an interview of Dr. Cordain. He mentions that Huntington Chorea seems to be an autoimmune disease. I have Huntington Chorea in my family. So obviously I’d like to know more about your or his findings. Could you please explain if there is any study showing this and what foods one should eliminate from the diet? I’d be so grateful.



Dr. Cordain’s Response:

Hi Tim,

Huntington’s Chorea or Huntington’s Disease (HD) results from lesions (alpha synuclein crosslinks) occurring in the brain which cause the characteristic symptoms (tremors and paralysis) of HD. It is well documented that a genetic basis underlies the development of HD. HD patients inherit a specific gene which causes increased expression of a protein called mutant huntingtin protein (mHTT). Whereas people without HD have inherited a gene which expresses the normal version of this protein, simply called, huntingtin protein (HTT). The over expression of mHTT at the expense of HTT is thought to cause the brain lesions of HD.

So, can diet have anything to do with whether or not a person with the mHTT genetic makeup goes on to develop the disease? Yes, and let me explain the underlying rationale. The imbalance in the mHTT to HTT ratio that occurs in HD patients requires the inheritance of the mHTT gene, however the gene cannot make its product without the presence of an enzyme called transglutaminase (TG). Transglutaminase is a post-translational enzyme (meaning that it is required to produce the gene product after the gene has been translated within a cell’s nucleus). TG is a ubiquitous post translational enzyme that is found throughout the body’s tissues, particularly in the gut, nervous tissue and brain. Without the presence of adequate concentrations of TG in the brain, mHTT cannot be produced in sufficient quantity to imbalance the mHtt to HHT ratio that results in HD.

So the $64,000 question in HD: what is the environmental trigger that causes over expression of TG in the brain? Plain and simple, it is wheat. More specifically, a storage protein in wheat called Gliadin. Unlike other dietary proteins, Gliadin is an unusual protein because it is resistant to the enzymes in the human gut (proteases) which normally degrade proteins into their constituent amino acids. Consequently, Gliadin arrives in the small intestine intact where it has recently been shown to bind a gut receptor (the CRX2 chemokine receptor). When Gliadin from wheat binds CRX2 it causes the intestinal cells to release a recently discovered enzyme known as zonulin. Zonulin release by gut cells causes the gut to become “leaky” and allow passage of intact proteins across the gut barrier — including Gliadin itself.

Once Gliadin bypasses the gut barrier, it is immediately catalyzed by transglutaminase (TG) which is expressed by local intestinal cells. When you eat wheat on a daily basis, there is so much dietary Gliadin bypassing the gut barrier that it overwhelms the ability of the intestinal cells to produce the enzyme (TG) to catalyze the substrate (Gliadin). Intestinal cells as well as all other cells in the body react to this overload of circulating Gliadin by up-regulating (increasing) TG production.

The proof of the pudding lies in the experimental evidence. Unfortunately no randomized controlled trials of Gliadin free diets in HD patients have been examined to date. Having said this, I am aware of a single HD patient in S. California who was a member of a local CrossFit Gym, and who had been diagnosed with HD by a group of University neurologists employing MRI technology to detect the characteristic brain lesions. After approximately 8 months following adoption of a Paleo Diet (Gliadin free), this patient experienced a dramatic reduction in disease symptoms and subsequent MRI evaluation indicated a reduction in lesion volume. In addition to HD, numerous ataxia patients respond favorably to Gliadin free diets.


Loren Cordain, Ph.D., Professor Emeritus

About Loren Cordain, PhD, Professor Emeritus

Loren Cordain, PhD, Professor EmeritusDr. Loren Cordain is Professor Emeritus of the Department of Health and Exercise Science at Colorado State University in Fort Collins, Colorado. His research emphasis over the past 20 years has focused upon the evolutionary and anthropological basis for diet, health and well being in modern humans. Dr. Cordain’s scientific publications have examined the nutritional characteristics of worldwide hunter-gatherer diets as well as the nutrient composition of wild plant and animal foods consumed by foraging humans. He is the world’s leading expert on Paleolithic diets and has lectured extensively on the Paleolithic nutrition worldwide. Dr. Cordain is the author of six popular bestselling books including The Real Paleo Diet Cookbook, The Paleo Diet, The Paleo Answer, and The Paleo Diet Cookbook, summarizing his research findings.

Comments to this website are moderated by our editorial board. For approval, comments need to be relevant to the article and free of profanities and personal attacks. We encourage cordial debates for the betterment of understanding and discovery. Comments that advertise or promote a business will also not be approved, however, links to relevant blog posts that follow the aforementioned criteria will be allowed. Thank you.

“4” Comments

  1. Wow..
    Ijust decided to look into the Paleo diet due to being diagnosed with Graves Disease.. but more excited in reading the article on Huntingtons..As my brother has this and i also am a gene carrier which i show no signs.. But now I am more intrigued to try this diet. Hoping to see big improvements in myself.. and perhaps a new diet for my dear brother..
    Thank you for this information 😀

  2. I was interested in reading the following article, but the link is not working. Can you fix please? The article…Energy-restricted diets based on a distinct food selection affecting the glycemic index induce different weight loss and oxidative response

Leave a Reply

Your email address will not be published. Required fields are marked *

Affiliates and Credentials