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:11/14/2016)... SARASOTA, Fla., Nov. 14, 2016  xG Technology, Inc. ... in providing critical wireless communications for use in challenging ... ended September 30, 2016. Management will hold a conference ... at 5:00 p.m. Eastern Time (details below). ... announced a $16 million binding agreement to acquire Vislink ...
(Date:6/27/2016)... -- Research and Markets has announced the addition of ... their offering. The report forecasts ... grow at a CAGR of 12.28% during the period 2016-2020. ... market analysis with inputs from industry experts. The report covers the ... report also includes a discussion of the key vendors operating in ...
(Date:6/22/2016)... , June 22, 2016   ... management and verification solutions, has partnered with ... software solutions for Visitor Management, Self-Service Kiosks ... provides products that add functional enhancements to ... provides corporations and venues with an automated ...
Breaking Biology News(10 mins):
(Date:12/2/2016)... PA (PRWEB) , ... December 01, 2016 , ... ... through industry-wide collaboration, standardization and a beautiful technology experience. All three tenets were on ... than 100 clinical trial leaders from over 40 sponsor, CRO and site organizations to ...
(Date:12/2/2016)...   SurePure, Inc. (OTCQB: SURP) a global ... has concluded an agreement with Tamarack Biotics under which ... to acquire units of the Company,s patented photopurification technology ... Concurrently with the option, SurePure has ... will seek regulatory approvals in the United ...
(Date:11/30/2016)... , Nov. 30, 2016  GenomOncology today announced the appointment ... of Medical Affairs.  Dr. Coleman will oversee ... company,s proprietary knowledge-enabled platform. The GenomOncology software suite empowers molecular ... sequencing data and clinical decision support, from quality control through ... , , ...
(Date:11/30/2016)... -- The Allen Institute for Cell Science has released ... collection of gene edited, fluorescently tagged human induced ... with unprecedented clarity. Distributed through the Coriell Institute ... crucial first step toward visualizing the dynamic organization ... cells healthy and what goes wrong in disease. ...
Breaking Biology Technology:
Cached News: