Navigation Links
New insights into how Huntington's disease attacks the brain

Scientific theory holds that Huntington's disease (HD) is caused by a mutant protein that arises within brain cells and kills them, triggering the genetic neurological disorder. Now a new UCLA Neuropsychiatric Institute study reveals the first strong evidence that the mutant protein also elicits toxic interactions from neighboring cells to provoke the fatal brain disorder. The May 5 edition of Neuron reports the findings.

"This is really important because most current disease models and drug development efforts rely on the assumption that Huntington's disease arises from within the target brain cells," explained Dr. William Yang, assistant professor at the UCLA Neuropsychiatric Institute and a member of the Brain Research Institute.

"Our model is the first to show that mutant HD proteins exert their influence on brain cells located near the target cells," he said. "These neighboring cells then interact with the target cells to spark disease."

To pinpoint the disorder's cellular origin, UCLA researchers developed two sets of mice with the human HD gene mutation. The first group was engineered to trigger production of the mutant HD protein throughout the brain. The second set of mice produced the mutant HD protein only in the target brain cells.

The scientists reasoned that if the mutant protein triggered the disease only from within the target cells, the second set of mice would display significant signs of the disorder. If HD required toxic interactions among cells throughout the brain, however, these same mice would show little or no signs of the disorder.

When comparing the two groups, the UCLA team discovered that the first set of mice demonstrated problems with motor control and showed visible degeneration of the target brain cells. In contrast, the second set of mice showed little signs of the disease.

"This is the first direct genetic evidence to demonstrate that abnormal interactions between cells can significant ly contribute to brain cell death in a living mouse model of Huntington's disease," said Yang.

Yang's team is now trying to pinpoint which of the neighboring cells generate Huntington's disease.

"Our next step will be determining how neighboring cells influence target cells and cause their death," he said. "Once we understand how these cells interact, the knowledge may lead to new therapeutic strategies to treat Huntington's disease."

Huntington's disease is a genetic brain disorder that usually strikes in mid-life, but can also attack the elderly and children as young as 2. Slowly depriving a person of their ability to think, speak, walk and swallow, the disease robs the person of their independence, leading to death within 10 to 25 years.

Every carrier of the HD gene mutation will develop the disease. Each child of a parent with Huntington's disease possesses a 50 percent risk of inheriting the illness. In the United States, the disease strikes 30,000 people and places another 150,000 persons at risk. The disorder affects males and females equally and crosses all ethnic and racial boundaries.


'"/>

Source:University of California - Los Angeles


Related biology news :

1. New insights into the software of life
2. Marsupial genome reveals insights into mammalian evolution
3. Frog’s ear canal may provide insights for understanding human hearing loss
4. New insights into neural tube defects
5. Engineered heart tissue offers insights into irregular heartbeats, defibrillator failure
6. AIDS vaccine research offers new insights on survival
7. Mutant mouse provides insights into breast cancer
8. From hot springs to rice farms, scientists reveal new insights into the secret lives of archaea
9. New insights into autoimmunity and depression
10. U. Iowa researchers improve Huntingtons disease symptoms in mice
11. Huntingtons cure in flies lays groundwork for broader treatment approaches
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:12/10/2018)... (PRWEB) , ... December 10, 2018 , ... ... a strategic alliance to advance their shared goal of accelerating the development of ... unique scale-up bioprocess systems to TRT’s core technology around human umbilical cord perivascular ...
(Date:12/5/2018)... ... December 03, 2018 , ... uBiome, the leader ... professor in the Division of Gastroenterology and Hepatology at UC San Diego and ... Board. Joining the board of leading scientists and doctors from around the world, ...
(Date:12/5/2018)... ... December 04, 2018 , ... ... the much-anticipated launch of their 100-liter (26 gallon) SAFEthread Drum. The 100-liter ... in 2017, which brought a much-needed modern redesign to the plastic drums being ...
Breaking Biology News(10 mins):
(Date:11/20/2018)... SPRINGS, Ga. (PRWEB) , ... November 19, 2018 , ... ... now receive custom dental implants from Dr. Dan Myers at THE DENTIST ... offering dental implant placement and restorations for the last 20 years. He now offers ...
(Date:11/20/2018)... , ... November 20, 2018 , ... Dr. Steven White ... for gingivitis at their office in Cornelius, NC. This common infection, left untreated, can ... in practice, along with special training and certification in laser dentistry, Drs. White and ...
(Date:11/13/2018)... ... November 13, 2018 , ... Renowned Honolulu veterinarian, Dr. Patrick Leadbeater, has ... Kauai. Dr. Leadbeater, of Kahala Pet Hospital on Oahu, is a ... began traveling to the island of Kauai on an almost weekly basis to perform ...
(Date:11/13/2018)... Sandy Springs, GA (PRWEB) , ... November 12, ... ... cosmetic dentist with state-of-the-art practices in Alpharetta and Sandy Springs, now welcomes ... clear aligners provide a discreet and comfortable alternative to traditional braces in the ...
Breaking Biology Technology: