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:7/29/2015)... ... , ... This year, the threat of flooding is higher in many areas ... water, mold is not far behind. Paul Davis, a leading provider of property restoration ... damage. Paul Davis is regularly called on to handle these types of emergencies, although ...
(Date:7/29/2015)... ... July 29, 2015 , ... CrossFit 269, offering a nationally ... Kalamazoo Strength & Conditioning. , The name change coincides with the release of ... training center over the past three years,” according to Jack Kelly, the center’s ...
(Date:7/29/2015)... ... July 29, 2015 , ... Orthopedic surgeons at Lourdes Medical Center of ... Rotator Cuff System, a new implant to treat a common source of shoulder pain ... regeneration of additional tendon tissue, which could allow for faster healing and a decreased ...
(Date:7/29/2015)... ... July 29, 2015 , ... Wedding season is in full swing, which means bridal beauty ... of looking good on the big day. Every bride wants to look and feel beautiful, ... , According to a recent study by the American Academy of ...
(Date:7/29/2015)... ... July 29, 2015 , ... Fastline Publications, ... of 2015 on RFD-TV. , Fastline is dedicated to quality agricultural advertising, both ... contains the largest and freshest searchable agriculture equipment database in the world. The ...
Breaking Medicine News(10 mins):Health News:Paul Davis Offers Tips to Minimize Property Damage and Health Risks This Flood Season 2Health News:Paul Davis Offers Tips to Minimize Property Damage and Health Risks This Flood Season 3Health News:Kalamazoo’s CrossFit 269 Changes Name, Opens New Website 2Health News:Kalamazoo’s CrossFit 269 Changes Name, Opens New Website 3Health News:Kalamazoo’s CrossFit 269 Changes Name, Opens New Website 4Health News:Lourdes Orthopedics First in NJ to Offer Bioinductive Implant for Rotator Cuff Repair 2Health News:Lourdes Orthopedics First in NJ to Offer Bioinductive Implant for Rotator Cuff Repair 3Health News:Say I Do To Surgery: Bridal Beauty On The Rise According to New Survey 2Health News:Say I Do To Surgery: Bridal Beauty On The Rise According to New Survey 3Health News:Fastline Publications to Partner with American Farmer in Upcoming Episode Airing on RFD-TV 2
(Date:7/29/2015)... Calif. , July 29, 2015 /PRNewswire/ ... or the "Company"), the leading provider of ... sciences industry, serving markets including immunotherapies, stem ... manufacturers, animal health, and reproductive medicine, announced ... sale of common stock and warrants (the "Units") under ...
(Date:7/29/2015)... 2015 Research and Markets ( http://www.researchandmarkets.com/research/sxwk89/3d_metrology ) ... Market by Product (CMM, ODS, and VMM), Application (Automotive, ... (North America, Europe, APAC, RoW) - Global Forecast to ... The 3D metrology market was valued at $1,456.7 ... a rate of 7.11% during the forecast period. ...
(Date:7/29/2015)... DIEGO , July 29, 2015  ViaCyte, Inc., a privately-held ... for the treatment of diabetes in clinical trials, today announced the ... STEP ONE, or S afety, T olerability, and ... One Diabetes.  The new site, which is the first in ... Alberta Hospitals in Edmonton , Alberta.  ...
Breaking Medicine Technology:Cryoport Completes $6.8 Million Equity Capital Raise 2Cryoport Completes $6.8 Million Equity Capital Raise 3Global 3D Metrology Market 2015-2020: Products (CMM, ODS, and VMM) & Applications (Automotive, Aerospace, Energy & Power, Medical and Electronics) 2ViaCyte Announces Second Clinical Trial Site at University of Alberta 2ViaCyte Announces Second Clinical Trial Site at University of Alberta 3ViaCyte Announces Second Clinical Trial Site at University of Alberta 4
Cached News: