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:4/26/2016)... 2016 Research and Markets has ... Market 2016-2020"  report to their offering.  , ,     ... The analysts forecast the global multimodal biometrics market ... the period 2016-2020.  Multimodal biometrics is ... as the healthcare, BFSI, transportation, automotive, and government ...
(Date:4/14/2016)... April 14, 2016 BioCatch ... Detection, today announced the appointment of Eyal Goldwerger ... role. Goldwerger,s leadership appointment comes at a ... of the deployment of its platform at several of ... technology, which discerns unique cognitive and physiological factors, is ...
(Date:3/29/2016)... , March 29, 2016 LegacyXChange, ... LegacyXChange "LEGX" and SelectaDNA/CSI Protect are pleased to announce ... used in a variety of writing instruments, ensuring athletes ... originally created collectibles from athletes on LegacyXChange will be ... of the DNA. Bill Bollander , ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... BEACH, Calif. , June 23, 2016  Blueprint ... new biological discoveries to the medical community, has closed ... co-founder Matthew Nunez . "We have ... us with the capital we need to meet our ... will essentially provide us the runway to complete validation ...
(Date:6/23/2016)... NEW YORK , June 23, 2016 ... the trading session at 4,833.32, down 0.22%; the Dow Jones ... the S&P 500 closed at 2,085.45, down 0.17%. Stock-Callers.com has ... INFI ), Nektar Therapeutics (NASDAQ: NKTR ... BIND Therapeutics Inc. (NASDAQ: BIND ). Learn more ...
(Date:6/23/2016)... ... June 23, 2016 , ... Regulatory Compliance ... consulting, provides a free webinar on Performing Quality Investigations: Getting to ... 12pm CT at no charge. , Incomplete investigations are still a major concern ...
(Date:6/23/2016)... FRANCISCO , June 22, 2016  Amgen (NASDAQ: ... sponsorship of the QB3@953 life sciences incubator ... human health. The shared laboratory space at QB3@953 was ... overcome a key obstacle for many early stage organizations ... part of the sponsorship, Amgen launched two "Amgen Golden ...
Breaking Biology Technology:
Cached News: