Working with genetically engineered mice, Johns Hopkins neuroscientists report they have identified what they believe is the cause of the vast disintegration of a part of the brain called the corpus striatum in rodents and people with Huntington’s disease: loss of the ability to make the amino acid cysteine. They also found that disease progression slowed in mice that were fed a diet rich in cysteine, which is found in foods such as wheat germ and whey protein.
Their results suggest further investigation into cysteine supplementation as a candidate therapeutic in people with the disease.
Up to 90 percent of the human corpus striatum, a brain structure that moderates mood, movement and cognition, degenerates in people with Huntington’s disease, a condition marked by widespread motor and intellectual disability. And while the genetic mutation underlying Huntington’s disease has long been known, the precise cause of that degeneration has remained a mystery.
In a report on their discovery in the advanced online publication of Nature on March 26, the Johns Hopkins researchers, led by Solomon Snyder, M.D., tracked the degenerative process to the absence of an enzyme, cystathionine gamma lyase, or CSE.
“Usually it’s very hard, if not impossible, to develop straightforward mechanisms that explain what’s going on in a disease. What’s even harder is even if you can find a mechanism that causes a tissue to rot, usually there’s nothing you can do about it,” says Snyder, a professor of neuroscience at the Johns Hopkins University School of Medicine. “In this case, there is.”
Huntington’s disease, an inherited disorder, does its damage because of abnormal DNA coding for the amino acid glutamine. Healthy individuals have some 15 to 20 DNA “repeats” in that part of their genetic code, while Huntington’s disease gene carriers have more than 36 — and often upward of 100. Children born to a parent carrier have a 50/50 chance of inheriting the disorder, and the greater the number of repeats, the earlier the age of onset of the incurable disorder.
Bindu Diana Paul, Ph.D., a molecular neuroscientist and faculty instructor in Snyder’s laboratory, was studying mice lacking CSE, which helps make the amino acid cysteine and hydrogen sulfide that moderate blood pressure and heart function. Paul, who had previous research experience with Huntington’s disease, says she was startled to observe that her mutant mice also behaved a lot like those with the disease.
When a normal mouse is dangled upside down from its tail, it will twist and turn and try to bite the offending hand, she explains. But her CSE-knockout mice stayed relatively still and clasped their paws together — the same behavior she’d observed in mice with the rodent equivalent of Huntington’s disease. “It looked like there was a neurological deficit,” Paul says. “But nobody had looked at CSE in the brain.”
Paul and Snyder began monitoring CSE in mouse and human brain tissues and found
http://www.thefrankmedrano.com/shop/getting-started-calisthenics-ebook ^^^Available NOW!! Getting Started CALISTHENICS E-BOOK!!! https://www.facebook.com/fra…
Video Rating: 4 / 5
CLASSIC STYLE WITH MODERN COMFORT The Nike Legend Poly Short Sleeve Workout Shirt for men delivers an ultra-lightweight feel and superior performance, taking it beyond basic to downright essential. Nike Dri-FIT 100% polyester fabric Dri-FIT fabric wicks away sweat to help keep you comfortable Short sleeve and short sleeve construction ideal for a variety of training conditions Neck tape added for durability and comfort Swoosh design upper left chest Size S Color Black
List Price: $ 24.95
Price: $ 24.95
Does your child get enough exercise? You can get your children more active with these 5 fun parenting tips.
In just four steps, you can improve your health for the better and this article will show you how. The steps below are bite-sized nuggets of health information you can actually use to live a healthy life. Here we go… Nutritional supplements are the first step As far as I’m concerned, they are very important […]
Several food and nutrition trends were in the spotlight this year, including the continued rise of plant-based diets, non-dairy ice creams and superfoods that are blasts from the past — legumes, apples and cabbage. It can be easy to experience nutrition whiplash in the New Year, but don’t be a victim. Get a jump-start on […]
If you want to lose weight, you’re not alone. More than half of Americans desire to shed pounds, according to Gallup. This goal inspires people to take action in many ways, from increasing exercise to modifying meals. One thing many people do is skip breakfast in order to lower calorie intake. While this may seem […]