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:12/10/2014)... been here before: you desperately need to sign into your online ... answer to your secret question. What,s your dog,s birthday? Who was ... , a digital infrastructure security company, launches the app ... with usernames, passwords and PINs – 1U TM . ...
(Date:12/10/2014)... Baptist Medical Center today announced plans for a new medical ... $50 million capital project is part of a larger capital ... The medical education building will be located in the ... 525@vine in Wake Forest Innovation Quarter. Construction will begin immediately ...
(Date:12/10/2014)... 08, 2014 Research and Markets ( http://www.researchandmarkets.com/research/xclcvw/biometrics_market ... Market in Japan 2014-2018" report to their offering. ... The integration of biometrics and smart cards for projects ... license is one of the major trends witnessed in ...
Breaking Biology News(10 mins):The Password is Finally Dead: Launch of 1U Mobile App Eliminates Need for All Usernames and Passwords 2The Password is Finally Dead: Launch of 1U Mobile App Eliminates Need for All Usernames and Passwords 3Wake Forest Baptist to Build New Medical Education Facility In Wake Forest Innovation Quarter 2Wake Forest Baptist to Build New Medical Education Facility In Wake Forest Innovation Quarter 3Wake Forest Baptist to Build New Medical Education Facility In Wake Forest Innovation Quarter 4Biometrics Market in Japan 2014-2018: Key Vendors are DDS, Fujitsu, Hitachi and NEC 2
(Date:1/22/2015)... 22, 2015 GEA Niro Soavi the ... laboratory homogenizer, the PandaPLUS 2000, which is ideal for ... and cell disruption . This compact laboratory homogenizer ... juices, liquid food, food additives and ingredients as well ...
(Date:1/22/2015)... 2015 Crystal Diagnostics (CDx) Xpress System, a ... received AOAC-PTM Certifications for the six non-O157 Shiga toxin-producing E. ... referred to as STEC or the “Big-6”) as well as ... (cfu) per 325 g of raw ground beef and raw ...
(Date:1/22/2015)... January 22, 2015 Selexis SA, a ... Research Cell Banks (RCBs) used for drug discovery to ... Banks will include Next-Generation Sequencing (NGS) data ... de-risks biologic manufacturing by ensuring the integrity of the ...
(Date:1/22/2015)... Jan. 22, 2015   GenoSpace , a precision medicine software ... enable the broad use of genomic, imaging and other biomedical ... of Michelle Munson , CEO of Aspera, an IBM ... Logo - http://photos.prnewswire.com/prnh/20150122/170713 "We are ...
Breaking Biology Technology:GEA Niro Soavi Announces the PandaPLUS 2000 Homogenizer for Processing New Applications for Nanoemulsions and Cell Disruption 2Crystal Diagnostics Awarded AOAC-PTM Accreditation for the Rapid Detection of “Big 6” E.coli Food Pathogens 2Selexis Generated Research Cell Banks Now Fully Sequenced Using Next-Generation Sequencing 2GenoSpace Expands Board with Appointment of Michelle Munson 2
Cached News: