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:
*Name:
*Comment:
*Email:


(Date:4/13/2017)... April 13, 2017 UBM,s Advanced Design and ... will feature emerging and evolving technology through its 3D ... will run alongside the expo portion of the event ... and demonstrations focused on trending topics within 3D printing ... and manufacturing event will take place June 13-15, 2017 at ...
(Date:4/11/2017)... -- NXT-ID, Inc. (NASDAQ:   NXTD ) ("NXT-ID" ... of independent Directors Mr. Robin D. Richards and ... furthering the company,s corporate governance and expertise. ... Gino Pereira , Chief Executive Officer ... guidance and benefiting from their considerable expertise as we move ...
(Date:4/4/2017)... , April 4, 2017   EyeLock LLC ... announced that the United States Patent and Trademark Office ... broadly covers the linking of an iris image with ... transaction) and represents the company,s 45 th issued ... patent is very timely given the multi-modal biometric capabilities ...
Breaking Biology News(10 mins):
(Date:4/21/2017)... ... April 21, 2017 , ... The ... the focus of researchers, engineers, product developers, and industry suppliers gathered last week ... by SPIE, the international society for optics and photonics , the event ...
(Date:4/20/2017)... , ... April 20, 2017 , ... ... , this new webinar will explore challenging patient cases when screening for direct ... hospital, there may be a need for bridging parental anticoagulation especially for those ...
(Date:4/20/2017)... ... April 20, 2017 , ... Husson ... clinical research community’s growing body of knowledge during its Eighth Annual Research ... and the adjacent Darling Atrium. During the event, undergraduates, graduate students, and faculty ...
(Date:4/20/2017)... ... April 20, 2017 , ... Parallel6™ , the leader ... announced today that they were named one of the 2017 Top 10 eClinical ... developments in the pharmaceutical industry. , “We take pride in honoring Parallel6 as one ...
Breaking Biology Technology:
Cached News: