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:8/15/2017)... Aug. 15 2017   ivWatch LLC , a medical device ... therapy, today announced receipt of its ISO 13485 Certification, the global ... International Organization for Standardization (ISO®). ... ivWatch Model 400 Continuous Monitoring device for the early detection of ... "This is an important milestone ...
(Date:7/20/2017)... DAL ) customers now can use fingerprints instead of their boarding ... ... biometrics to board aircraft at Reagan Washington National Airport ... Delta,s biometric boarding pass experience that launched in May at the ... to allow eligible Delta SkyMiles Members who are enrolled in CLEAR to ...
(Date:6/30/2017)... 30, 2017 Today, American Trucking Associations ... of face and eye tracking software, became the ... program. "Artificial intelligence and advanced ... monitor a driver,s attentiveness levels while on the ... to detect fatigue and prevent potential accidents, which ...
Breaking Biology News(10 mins):
(Date:8/18/2017)... ... August 18, 2017 , ... ... will feature Federal Hybrids, Inc. in an upcoming episode, scheduled to broadcast fourth ... Farmer will explore Federal Hybrids, the independent, family-owned seed company. Educating audiences about ...
(Date:8/18/2017)... ... August 18, 2017 , ... OAI, a ... MEMS, and Microfluidics Industries, announces the new Model 800E front and backside, semi-automatic ... automated production mask aligners. OAI has already received and installed several orders ...
(Date:8/17/2017)... ... August 17, 2017 , ... ... market news outlet had provided a research update on Aytu Bioscience and cited ... product. , According to Soulstring, prescription rates for Natesto® have more than doubled ...
(Date:8/17/2017)... ... August 17, 2017 , ... Cynvenio Biosystems, Inc., a leader ... launch of a new breast cancer monitoring study in partnership with Saint Luke’s ... potential for early detection of recurrent breast cancer using LiquidBiopsy and natural killer ...
Breaking Biology Technology:
Cached News: