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:8/31/2018)... ... August 30, 2018 , ... ... a complimentary cryogenic freezer program for new clients. The freezers are very convenient ... biologics from R3 Stem Cell consist of amniotic and umbilical cord tissue. They ...
(Date:8/29/2018)... (PRWEB) , ... August 28, 2018 , ... ... and cloud infrastructure automation software, announced that Data In Science Technologies has implemented ... HudsonAlpha Institute for Biotechnology is a non-profit genomics and genetics research institute ...
(Date:8/26/2018)... (PRWEB) , ... August 24, ... ... become an essential tool in clinical diagnostic laboratories. However, this technology introduces ... at Guy’s Hospital London, which leads the South London NHS Genomic Medicine ...
Breaking Biology News(10 mins):
(Date:9/7/2018)... ... 2018 , ... Flagship Biosciences’ MuscleMap algorithm that supports therapeutic ... effective as manual pathology. The study, “Validation of a Muscle-Specific Tissue Image-Analysis Tool ... the Archives of Pathology & Laboratory Medicine on August 31, 2018. Download the ...
(Date:9/1/2018)... ... August 31, 2018 , ... ... the impact of a proprietary biofield energy treated nutraceutical supplement used in combating ... immune function response and blood, as well as, organ-specific inflammation. The reported data ...
(Date:8/31/2018)... ... August 30, 2018 , ... Small, wireless and networked ... slimming down and getting connected, too. Draper’s latest system is tiny in size, but ... , A driving factor in the new implant design is the growing awareness that ...
(Date:8/29/2018)... ... ... Cognition Corporation ®, a software company specializing in medical device compliance and ... Ryan Ward, Director of Engineering at Zimmer Biomet, has been announced as a speaker ... Zimmer Biomet for almost 15 years, Mr. Ward will focus his talk about how ...
Breaking Biology Technology: