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:8/11/2015)... Today, ZUK announced its Android smartphone Z1, featuring FPC,s ... in 2015 that relate to sales of FPC1155 for this smartphone ... MSEK for 2015. Jörgen Lantto, CEO of FPC, comments: ... smartphone manufacturer in China and we are ... for Z1 , its ...
(Date:8/6/2015)... Germany , August 6, 2015 ... Instruments (SMI) shows the world,s first Eye ... on Epson,s Moverio BT-200 see-through head mounted display and ... solution, unprecedented quality and efficiency is brought to personalized ... For the first time, professionals and researchers can integrate ...
(Date:8/5/2015)... 2015 The biosensors market is proving highly ... into newer sectors, and development of devices resulting in ... has seen the entry of multiple participants each year ... (Photo - http://photos.prnewswire.com/prnh/20150805/255570 ) ... the Global Biosensors Market ( http://www.frost.com/nee9 ), finds ...
Breaking Biology News(10 mins):FPC's Touch Fingerprint Sensor FPC1155 in ZUK's Smartphone Z1 2SMI Shows First Eye Tracking Upgrade for Augmented Reality Glasses 2SMI Shows First Eye Tracking Upgrade for Augmented Reality Glasses 3Innovative Biosensors Incite Use in Non-Traditional Applications 2Innovative Biosensors Incite Use in Non-Traditional Applications 3Innovative Biosensors Incite Use in Non-Traditional Applications 4
(Date:8/26/2015)... , Aug. 26, 2015  Roka Bioscience, Inc. (NASDAQ: ... providing advanced testing solutions for the detection of foodborne ... present at the Sidoti & Company Emerging Growth Conference ... be held at the New York Marriott Marquis. ... be available through the investor relations section of Roka ...
(Date:8/26/2015)... FRANCISCO , Aug. 26, 2015  Platform ... that it has received a $1.5M Phase II ... (NIH) to further develop its therapeutic agent to ... clinical need in end stage renal disease (ESRD) ... the National Institute of Diabetes and Digestive and ...
(Date:8/26/2015)... HONG KONG , Aug. 26, 2015 ... "Company"), China,s leading provider of ... and stem cell storage services, today announced that the ... Holdings Limited ("MO2"), an entity wholly owned by Mr. ... the Company, has acquired the outstanding shares (the "Shares") ...
(Date:8/26/2015)... ... August 26, 2015 , ... PRC Clinical, ... new business relationships with local sponsors in southern California. The Clinical Trial Management Expert ... development processes and regulatory pathways., , Stem Cell Meeting ...
Breaking Biology Technology:Symic Biomedical Awarded $1.5MM NIH Phase II SBIR Grant to Develop its Proteoglycan Mimetic Therapeutics to Reduce Vascular Access Failures in ESRD 2Symic Biomedical Awarded $1.5MM NIH Phase II SBIR Grant to Develop its Proteoglycan Mimetic Therapeutics to Reduce Vascular Access Failures in ESRD 3China Cord Blood Corporation Receives Notice of Transactions Regarding its 7% Senior Convertible Notes Due 2017 2China Cord Blood Corporation Receives Notice of Transactions Regarding its 7% Senior Convertible Notes Due 2017 3PRC Clinical To Attend Major Industry Conferences And Meet Sponsor Companies In The San Diego Biotech Hub 2PRC Clinical To Attend Major Industry Conferences And Meet Sponsor Companies In The San Diego Biotech Hub 3
Cached News: