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:3/27/2021)... ... March 25, 2021 , ... Phlexglobal announced ... Planet Group, has selected Phlexglobal and its innovative regulatory SaaS software, PhlexSubmission, as ... a comprehensive review of five regulatory software companies, with the review team including ...
(Date:3/27/2021)... ... 25, 2021 , ... The 2021 Virtual Conference on Clinical Trial Supply-Europe ... More and more, clinical trial supply conferences are featuring speakers and forums that ... Asymmetrex’s founder and CEO, James L. Sherley, M.D., Ph.D., presented a talk ...
(Date:3/23/2021)... ... March 23, 2021 , ... G-CON Manufacturing (G-CON), the ... by Matica Biotechnology (Matica Bio), a contract development and manufacturing organization (CDMO) specializing ... the cleanroom build out for its new GMP production facility in College Station, ...
Breaking Biology News(10 mins):
(Date:3/27/2021)... ... March 24, 2021 , ... ... Life Sciences and Healthcare firms of all sizes, adds depth to its team ... specialist. Pardillo, who earned his doctorate in computational chemistry from Florida International University ...
(Date:3/27/2021)... ... March 24, 2021 , ... ABI Wellness, ... and reporting approach designed under CEO Mark Watson, today announced a webinar dedicated ... featuring guest speakers Dr. Cameron Clark, Neuropsychologist and Founder of Sharp Thinking, and ...
(Date:3/27/2021)... ... ... The Xtalks editorial team is pleased to announce the launch of the ... joined by editorial team members Ayesha Rashid, Sydney Perelmutter and Mira Nabulsi to discuss ... including insights from industry experts. , The Xtalks Life Science podcast will feature ...
(Date:3/23/2021)... Conn. (PRWEB) , ... March 23, 2021 , ... ... develops solutions for characterizing microbiome populations down to the strain level, recently unveiled ... applications. , Not all microbes are created equal: some are easy to ...
Breaking Biology Technology:
Cached News: