Navigation Links
New findings on protein misfolding
Date:9/18/2012

Misfolded proteins can cause various neurodegenerative diseases such as spinocerebellar ataxias (SCAs) or Huntington's disease, which are characterized by a progressive loss of neurons in the brain. Researchers of the Max Delbrck Center for Molecular Medicine (MDC) Berlin-Buch, Germany, together with their colleagues of the Universit Paris Diderot, Paris, France, have now identified 21 proteins that specifically bind to a protein called ataxin-1. Twelve of these proteins enhance the misfolding of ataxin-1 and thus promote the formation of harmful protein aggregate structures, whereas nine of them prevent the misfolding (PLoS Genetics, doi: 10.1371/journal.pgen.1002897)*.

Proteins only function properly when the chains of amino acids, from which they are built, fold correctly. Misfolded proteins can be toxic for the cells and assemble into insoluble aggregates together with other proteins. Ataxin-1, the protein that the researchers have now investigated, is very prone to misfolding due to inherited gene defects that cause neurodegenerative diseases. The reason for this is that the amino acid glutamine is repeated in the amino acid chain of ataxin-1 very often - the more glutamine, the more toxic the protein. Approximately 40 repeats of glutamine are considered to be toxic for the cells.

Now, Dr. Spyros Petrakis, Dr. Miguel Andrade, Professor Erich Wanker and colleagues have identified 21 proteins that mainly interact with ataxin-1 and influence its folding or misfolding. Twelve of these proteins enhance the toxicity of ataxin-1 for the nerve cells, whereas nine of the identified proteins reduce its toxicity.

Furthermore, the researchers detected a common feature in the structure of those proteins that enhances toxicity and aggregation. It is a special structure scientists call "coiled-coil-domain" because it resembles a double twisted spiral or helix. Apparently this structure promotes aggregation, because proteins that interact with ataxin-1 and have this domain enhance the toxic effect of mutated ataxin-1. As the researchers said, this structure could be a potential target for therapy: "A careful analysis of the molecular details could help to discover drugs that suppress toxic processes."


'/>"/>

Contact: Bachtler, Barbara
bachtler@mdc-berlin.de
49-309-406-3896
Helmholtz Association of German Research Centres
Source:Eurekalert

Related biology news :

1. Blue Ribbon Panel unveils findings on logistical improvements to support Antarctic science
2. ACRG and BGI report findings from genomics research on recurrent hepatitis B virus integration
3. Chemists develop reversible method of tagging proteins
4. Anchoring proteins influence glucose metabolism and insulin release
5. Research yields two firsts regarding protein crucial to human cardiac function
6. Too much protein HUWE1 causes intellectual disability
7. Hulk protein, Grb10, controls muscle growth
8. Collagen-seeking synthetic protein could lead doctors to tumor locations
9. A new look at proteins in living cells
10. Low oxygen levels may decrease life-saving protein in spinal muscular atrophy
11. Molecular and protein markers discovered for liver transplant failure from hepatitis C
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:1/18/2016)... 2016  Extenua Inc., a pioneering developer of ... and access of ubiquitous on-premise and cloud storage, today ... Cyber.  ... C4ISR and Cyber initiatives in support of National ... technology solutions," said Steve Visconti , Extenua ...
(Date:1/13/2016)... January 13, 2016 ... addition of the  "India Biometrics Authentication ... Forecast (2015-2020)"  report to their ... has announced the addition of the  ... - Estimation & Forecast (2015-2020)" ...
(Date:1/8/2016)... and MANCHESTER, United Kingdom , Jan. 8, ... of innovative sensor-based diagnostic products, today announced the closing of ... and existing investors.  Proceeds from the financing will be used ... a hand-held device for detecting early-stage pressure ulcers. ... after receiving CE Mark approval. The device,s introduction ...
Breaking Biology News(10 mins):
(Date:2/3/2016)... BRUNSWICK, N.J. , Feb. 3, 2016 /PRNewswire-USNewswire/ ... grants totaling more than $1 million for researchers ... are working on health-related research that demonstrates exciting ... this round of funding for the New Jersey ... for faculty members at these educational institutions— Princeton ...
(Date:2/3/2016)... , ... February 03, 2016 , ... ... a series of potential targets (epitopes) specific to misfolded, propagating strains of Amyloid ... create specific monoclonal antibody therapeutics for Alzheimer’s. , Following on from the first ...
(Date:2/3/2016)... ... 2016 , ... Resilinc released online today its ... 750 unique supply chain notifications and alerts generated by its EventWatch ® ... management practitioners subscribe to the EventWatch service to receive early warnings and analysis ...
(Date:2/3/2016)... Calif. , Feb. 3, 2016  Today, Symphony ... of AlphaImpactRx , a leading provider of primary ... companies to IMS Health , a global information ... complementary offerings, capabilities and technologies will be integrated into ... growing global primary market research capabilities. ...
Breaking Biology Technology: