HOME >> BIOLOGY >> NEWS
Single Mechanism Seen To Underlie Group Of Neurodegenerative Diseases: Mutant Proteins Form Insoluble Masses In The Nuclei Of Neurons

Researchers at the University of Pennsylvania Medical Center and other institutions are converging on an understanding of the underlying mechanism responsible for more than a half-dozen debilitating neurodegenerative diseases. Among the movement disorders involved are Huntington's disease and Machado-Joseph disease, less well known than Huntington's but perhaps as common. The findings will likely guide new investigations into the neuropathological course of these diseases, as well as the search for possible interventions into their progression. Several related reports will appear in the August issue of Neuron and the August 8 issue of Cell.

Each of these diseases can be traced to a different flawed gene, but each shares a common problem in its DNA -- instructions for producing the amino acid glutamine are repeated excessively along a given stretch of a gene coding for an important protein. In normal proteins, the sequence of three nucleotides that constitutes glutamine -- cytosine, adenine, and guanine, or CAG -- is often repeated 15 to 20 times, but in the mutant proteins the repeats increase in number to 50 or even 100, with earlier onset and greater severity of the particular disease linked to higher numbers of the trinucleotide repeats. The cause for the increased repeats is not known.

Now, scientists have discovered that the proteins with glutamine expansions, which appear to function well in most respects, have a new and dangerous characteristic that their normal counterparts do not. In some neurons, the CAG-repeat proteins accumulate in the nuclei of the cells, aggregating into insoluble masses that can crowd and eventually fill much of the nuclear space. And once this aggregation has begun, the mutant proteins appear to recruit normal versions of the protein into the growing mass, called an inclusion, further exacerbating the problem.

"These aggregated proteins take over a significant fraction of the space in the nucleus and
'"/>

Contact: Franklin Hoke
hokef@mail.med.upenn.edu
215-349-5659
University of Pennsylvania School of Medicine
7-Aug-1997


Page: 1 2 3

Related biology news :

1. Single isolated mouse skin cell can generate into variety of epidermal tissues
2. Single gene mutation muddying Parkinsons risk forecasts
3. Single gene controls leaf form
4. Single molecules observed
5. Single gene leap led to flea-borne transmission of plague bacterium
6. Single cell type seems to control internal clock and pupil of eye
7. Single enzyme may be linked to obesity
8. Single virus tracing sets the stage for new infection-fighting drugs and gene-therapy strategies, Science authors say
9. Single amino acid mutation dramatically alters direction of a molecular motor
10. Single switch triggers two immune system genes
11. Single amino acid change in herpes virus prevents it from infecting neurons

Post Your Comments:
(Date:4/20/2015)... Calif. , April 20, 2015 Huntington ... Gabriel Valley to implant a new miniaturized, wireless monitoring ... System is the first and only FDA-approved heart failure ... hospital admissions when used by physicians to manage heart ... sensor that is implanted in the pulmonary artery (PA) ...
(Date:4/17/2015)... Increasing adoption in ... to drive biometric systems market in ... According to a recently released TechSci Research ... biometric systems market in Saudi Arabia ... CAGR of over 22% through 2020. The market ...
(Date:4/14/2015)... NXT-ID, Inc. (NASDAQ: NXTD ) ("NXT-ID" ... the growing mobile commerce market, announces that its Wocket® ... York Design Awards under the category  ,Product Design – ... Awards are part of a global multi-disciplinary awards program. ... the marketplace, industry and judging panel. Winners will be ...
Breaking Biology News(10 mins):Huntington Memorial Hospital Becomes First in the San Gabriel Valley to Offer Patients a New Heart Failure Monitoring Solution 2Huntington Memorial Hospital Becomes First in the San Gabriel Valley to Offer Patients a New Heart Failure Monitoring Solution 3Saudi Arabia Biometric Systems Market to Grow at 22% Through 2020, Says TechSci Research 2Saudi Arabia Biometric Systems Market to Grow at 22% Through 2020, Says TechSci Research 3NXT-ID's Wocket Smart Wallet Nominated for 2015 New York Design Awards in 'Product Design- Technology' 2NXT-ID's Wocket Smart Wallet Nominated for 2015 New York Design Awards in 'Product Design- Technology' 3
(Date:4/23/2015)... , April 23, 2015 BioTE Medical ... his book "Age Healthier Live Happier - Avoiding Over-Medication ... the Age Management Medicine Group Conference at JW Marriott ... 2015. ",Age Healthier Live Happier, is a ... avoid over-medication and searching for a higher quality of ...
(Date:4/23/2015)... 23, 2015 Follow us ... a series of genetic tests that determine individual ... Gehrig's disease, Huntington's disease, and Alzheimer's disease. Predictive ... composition and interaction in diseased tissue/cells to understand ... as the response to treatment, such as in ...
(Date:4/23/2015)... 23, 2015 Glemser Technologies ... first EMC® Enterprise Content Division (ECD) Certified Solution ... EMC Documentum® for Life Sciences Solution Suite . ... and capabilities to provide life sciences companies of ... , The EMC Documentum Solution Suite offers four ...
(Date:4/23/2015)... 23, 2015 /PRNewswire/ - Crystal Research Associates, LLC announced ... ® (EIO) on Aeterna Zentaris Inc. (NASDAQ: ... on Crystal Research Associates, website at www.crystalra.com ... at www.aezsinc.com . Aeterna Zentaris ("Aeterna" ... company in oncology, endocrinology, and women,s health. With a ...
Breaking Biology Technology:Release and Book Signing of BioTE Medical President Gary Donovitz M.D. 'Age Healthier Live Happier' at Age Management Medicine Group (AMMG) Conference 2Release and Book Signing of BioTE Medical President Gary Donovitz M.D. 'Age Healthier Live Happier' at Age Management Medicine Group (AMMG) Conference 3Increasing Disease Prevalence & Focus on Preventive Healthcare Drive the Predictive Diagnostics Market, According to a New Report by Global Industry Analysts, Inc. 2Increasing Disease Prevalence & Focus on Preventive Healthcare Drive the Predictive Diagnostics Market, According to a New Report by Global Industry Analysts, Inc. 3Glemser Earns Accreditation for Life Sciences Solution Suite 2Crystal Research Associates, LLC Issues Executive Informational Overview (EIO) on Aeterna Zentaris Inc. 2Crystal Research Associates, LLC Issues Executive Informational Overview (EIO) on Aeterna Zentaris Inc. 3
Cached News: