HOME >> MEDICINE >> NEWS
Study identifies possible mechanism for brain damage in Huntington's disease

Researchers from the MassGeneral Institute for Neurodegenerative Disease (MIND) have identified a possible mechanism underlying how the gene mutation that causes Huntington's disease (HD) leads to the degeneration and death of brain cells. In the Oct. 6 issue of Cell, they show that the abnormal form of the huntingtin protein, the product of the HD gene mutation, interferes with the production of a protein critical to cellular energy metabolism. The discovery is the first to bring together two processes believed to be involved in the pathology of HD the conversion of genetic information into proteins and the production of energy within cells.

"Our study indicates that these two pathogenic mechanisms are linked, in that disruption of gene transcription by mutant huntingtin leads to abnormal energy metabolism, which affects energy-dependent cellular processes and results in neurodegeneration," says Dimitri Krainc, MD, PhD, of MIND and the MGH Department of Neurology, who led the research team. "The role of mitochondria [subcellular structures that produce the cells' energy] in the process of nerve cell dysfunction and death is an emerging theme in neurodegenerative disorders, but the mechanism behind HD has been elusive."

HD causes the degeneration and death of cells in the basal ganglia an area deep within the brain particularly in a structure called the striatum. Although the precise function of the huntingtin protein is still unknown, recent studies have suggested that the mutant form directly interferes with transcription of neuronal genes. Evidence also has pointed to disruptions in cellular energy metabolism as key factors in HD. As a result, the MIND team focused on a protein called PGC-1a, which is known to regulate energy in cells throughout the body. Their previous research had shown that mice in which the PGC-1a gene had been knocked out developed brain lesions in the striatum.

To investigate the possible effect of th
'"/>

Contact: Sue McGreevey
smcgreevey@partners.org
617-724-2764
Massachusetts General Hospital
5-Oct-2006


Page: 1 2 3

Related medicine news :

1. Study, meta-analysis examine factors associated with death from heatstroke
2. Study says COPD testing is not measuring up
3. Study suggests loss of 2 types of neurons -- not just 1 -- triggers Parkinsons symptoms
4. Study reveals gaps in vaccine financing for underinsured children
5. Study suggests nonpharmaceutical interventions may be helpful in severe influenza outbreaks
6. Study shows radiofrequency ablation highly effective in treating kidney tumors
7. Study says normal but out-of-control enzyme may be culprit that signals some cells to become cancer
8. Study finds HIV protease inhibitor drugs may adversely affect the scaffolding of the cell nucleus
9. Study outlines how stroke, head injury can increase risk of Alzheimers disease
10. Study identifies new regulator of fat metabolism
11. Study shows Diachrome improves blood sugar control in people with type 2 diabetes

Post Your Comments:
(Date:11/24/2014)... 2014 The typical American diet includes ... ingredients. Consistently consuming a diet high in calories and ... excess fat that typically accumulate around the internal organs ... ugly head in the form of love handles, beer ... the decision to rid themselves of the burden of ...
(Date:11/24/2014)... CO, and Philadelphia, PA (PRWEB) November 24, 2014 ... provider of care coordination and disease management services ... has chosen to implement Essette’s Medical Management software ... new Essette SaaS platform in a program to ... Essette suite includes Population Health Management, Care Management, ...
(Date:11/24/2014)... San Diego, California (PRWEB) November 24, 2014 ... the San Diego Fertility Center, recently appeared on NBC to ... preservation option. Egg freezing has been trending in the news ... covering this expensive procedure for women. , “[The ... the women who are on a career hot path.” Says ...
(Date:11/23/2014)... The clinical trial report, "Aspergillosis ... on the Aspergillosis clinical trial scenario. This report ... clinical trials on Aspergillosis. It includes an overview ... as per the site of trial conduction across ... of disease clinical trials by their phase, trial ...
(Date:11/23/2014)... For the coming special Friday, Tidebuy, a ... great promotion for many products, especially the beautiful colored ... best choice with amazing discounts, up to 90% off. ... It is easy to buy colored wedding dresses in ... is also famous for delicate craftsmanship, elegant style and ...
Breaking Medicine News(10 mins):Health News:Ketogenic Diet is the Perfect Solution for Diet Doc Patients Who Want to Learn How to Lose Weight over the Holidays 2Health News:Ketogenic Diet is the Perfect Solution for Diet Doc Patients Who Want to Learn How to Lose Weight over the Holidays 3Health News:Ketogenic Diet is the Perfect Solution for Diet Doc Patients Who Want to Learn How to Lose Weight over the Holidays 4Health News:ReAdmission Solutions, LLC Selects Essette 2Health News:Dr. Michael Kettel of San Diego Fertility Center Gives “An Inside Look at Freezing Eggs” on NBC 2Health News:Dr. Michael Kettel of San Diego Fertility Center Gives “An Inside Look at Freezing Eggs” on NBC 3Health News:Aspergillosis Pipeline and Clinical Trials Market (Compnies and Drugs) Review H2 2014 Report at ReportsnReports.com 2Health News:Aspergillosis Pipeline and Clinical Trials Market (Compnies and Drugs) Review H2 2014 Report at ReportsnReports.com 3Health News:Aspergillosis Pipeline and Clinical Trials Market (Compnies and Drugs) Review H2 2014 Report at ReportsnReports.com 4Health News:Beautiful Wedding Dresses Available at Popular B2C Company Tidebuy 2
(Date:11/22/2014)... Statistics released from a number of organizations ... during the holidays, beginning with Thanksgiving eve, which is ... The data, highlighted in an Infographic ... of their annual Sober Days for the Holidays ... drinking and increased rates of DUI injuries and deaths ...
(Date:11/22/2014)... 2014 Many power shifts are occurring ... of patients in the biopharmaceutical market is impacting ... than ever, understanding how to effectively acquire, convert, ... essential. According to recent research ... spent about two-thirds of their patient-focused dollars on ...
(Date:11/21/2014)... DIEGO , Nov. 21, 2014  Tandem ... TNDM ), a medical device company and manufacturer ... announced a partnership with Tidepool, a non-profit dedicated ... open source software platform that increases diabetes data ... Tidepool Platform, which is currently under development, to ...
Breaking Medicine Technology:Alarming Stats Show Holiday Drinking an Issue for Many 2New Report Details Importance of Adopting a "Patient Journey" Framework in Marketing 2Tandem Diabetes Care Announces Partnership with Tidepool to Further Expand Access to Diabetes Device Data 2Tandem Diabetes Care Announces Partnership with Tidepool to Further Expand Access to Diabetes Device Data 3
Cached News: