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:2/21/2017)... PORTLAND, Ore. , Feb. 22, 2017  IBM ... Companies (Avamere Health Services, Infinity Rehab, Signature Hospice, Home ... that will apply the power of IBM cognitive computing ... health centers. By analyzing data streaming from sensors in ... physical and environmental conditions, and obtain deeper learnings into ...
(Date:2/13/2017)... WASHINGTON , Feb. 13, 2017 Former ... U.S. Senate Judiciary Committee, Janice Kephart of ... regarding President Donald Trump,s "Executive Order: Protecting ... States" (Jan. 27, 2017):  "As President Trump,s ... 9th Circuit has now essentially banned the travel ban, ...
(Date:2/8/2017)... Feb. 7, 2017 Report Highlights ... The global synthetic-biology market ... billion by 2021, growing at a compound annual growth rate ... overview of the global markets for synthetic biology. - Analyses ... 2016, and projections of compound annual growth rates (CAGRs) through ...
Breaking Biology News(10 mins):
(Date:2/22/2017)... ... February 22, 2017 , ... LabRoots , the leading ... the world, is pleased to announce the 2nd annual Precision Medicine Virtual Conference. ... online-only conference focused on the development and advancements in precision medicine. , Precision ...
(Date:2/22/2017)... MOINES, Iowa , Feb. 22, 2017 Origin (Origin ... agricultural biotechnology trait and seed provider, and Arcadia ... -based company that develops and commercializes agricultural productivity traits and nutritional ... a key corn biotechnology product developed in China ... of global regulatory trials. ...
(Date:2/22/2017)... ... February 22, 2017 , ... Pharma and biotech consulting firm ... director. Operating from Pennside’s Zurich headquarters, Pennside Partners, GmbH, Mr. Perkins brings 14 ... more than a decade with leading market research firm, GfK. He began his ...
(Date:2/21/2017)... 21, 2017 Synthetic Biologics, Inc. (NYSE MKT: ... microbiome to protect and restore the health of patients, intends to ... 31, 2016 on Thursday, March 2, 2017, and to host a ... information for the call is as follows: U.S. (toll ... ...
Breaking Biology Technology: