Featured Research

from universities, journals, and other organizations

Misplaced molecules: New insights into the causes of dementia

Date:
March 2, 2013
Source:
Helmholtz Association of German Research Centres
Summary:
Researchers have succeeded in gaining new insights into the causes of certain movement disorders and forms of dementia. Scientists were able to reproduce disease symptoms typical of amyotrophic lateral sclerosis and frontotemporal dementia in zebrafish.

A shortage of a protein called TDP-43 caused muscle wasting and stunted nerve cells. This finding supports the idea that malfunction of this protein plays a decisive role in ALS and FTD. The study is published in the Proceedings of the National Academy of Sciences (PNAS).

ALS is an incurable neurological disease which manifests as rapidly progressing muscle wasting. Both limbs and respiratory muscles are affected. This leads to impaired mobility and breathing problems. Patients commonly die within a few years after the symptoms emerged. In rare cases, of which the British physicist Stephen Hawking is the most notable, patients can live with the disease for a long time. In Germany estimates show over 150,000 patients suffering from ALS -- an average of 1 in 500 people.

Proteins gone astray

Over the last few years, there has been increasing evidence that ALS and FTD -- a form of dementia associated with changes in personality and social behaviour -- may have similar or even the same origins. The symptoms overlap and common factors have also been found at the microscopic level. In many cases, particles accumulate and form clumps in the patient's nerve cells: this applies particularly to the TDP-43 protein.

"Normally, this protein is located in the cell nucleus and is involved in processing genetic information," explains molecular biologist Dr. Bettina Schmid, who works at the DZNE Munich site and at LMU. "However, in cases of disease, TDP-43 accumulates outside the nucleus forming aggregates." Schmid explains that it is not yet clear whether these clumps are harmful. "However, the protein's normal function is clearly disrupted. It no longer reaches the nucleus to perform its actual task. There seems to be a relationship between this malfunction and the disease."

Studies on zebrafish

However, until now little was known about the function of TDP-43. What are the consequences when this protein becomes non-functional? In order to answer this question, the team led by Bettina Schmid cooperated with the research group of Prof. Christian Haass to investigate the larvae of specially bred zebrafish. Their genetic code had been modified in such a way that no TDP-43 was produced in the organism of the fish. The result: the young fish showed massive muscle wasting and died a few days after hatching. Moreover, the extensions of the nerve cells which control the muscles were abnormal.

"To some extent, these are symptoms typical of ALS and FTD. Therefore, a loss of function of TDP-43 does seem to play a critical role in the disease," says Haass, Site Speaker of the DZNE Munich Site and chair of Metabolic Biochemistry at LMU.

The study revealed one more finding which surprised the researchers: the blood flow of the fish was massively disturbed. "It is well known that circulatory disorders play a part in other forms of dementia, notably in the case of Alzheimer's," says Haass. "We now want to investigate whether such problems with blood flow may be a general problem of neurodegenerative diseases and whether such problems occur particularly in patients with ALS and FTD."


Story Source:

The above story is based on materials provided by Helmholtz Association of German Research Centres. Note: Materials may be edited for content and length.


Journal Reference:

  1. B. Schmid, A. Hruscha, S. Hogl, J. Banzhaf-Strathmann, K. Strecker, J. van der Zee, M. Teucke, S. Eimer, J. Hegermann, M. Kittelmann, E. Kremmer, M. Cruts, B. Solchenberger, L. Hasenkamp, F. van Bebber, C. Van Broeckhoven, D. Edbauer, S. F. Lichtenthaler, C. Haass. Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth. Proceedings of the National Academy of Sciences, 2013; DOI: 10.1073/pnas.1218311110

Cite This Page:

Helmholtz Association of German Research Centres. "Misplaced molecules: New insights into the causes of dementia." ScienceDaily. ScienceDaily, 2 March 2013. <www.sciencedaily.com/releases/2013/03/130302125402.htm>.
Helmholtz Association of German Research Centres. (2013, March 2). Misplaced molecules: New insights into the causes of dementia. ScienceDaily. Retrieved October 21, 2014 from www.sciencedaily.com/releases/2013/03/130302125402.htm
Helmholtz Association of German Research Centres. "Misplaced molecules: New insights into the causes of dementia." ScienceDaily. www.sciencedaily.com/releases/2013/03/130302125402.htm (accessed October 21, 2014).

Share This



More Mind & Brain News

Tuesday, October 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

First-Of-Its-Kind Treatment Gives Man Ability To Walk Again

First-Of-Its-Kind Treatment Gives Man Ability To Walk Again

Newsy (Oct. 21, 2014) A medical team has for the first time given a man the ability to walk again after transplanting cells from his brain onto his severed spinal cord. Video provided by Newsy
Powered by NewsLook.com
Portable Breathalyzer Gets You Home Safely

Portable Breathalyzer Gets You Home Safely

Buzz60 (Oct. 21, 2014) Breeze, a portable breathalyzer, gets you home safely by instantly showing your blood alcohol content, and with one tap, lets you call an Uber, a cab or a friend from your contact list to pick you up. Sean Dowling (@SeanDowlingTV) has the details. Video provided by Buzz60
Powered by NewsLook.com
Your Birth Season Might Determine Your Temperament

Your Birth Season Might Determine Your Temperament

Newsy (Oct. 20, 2014) A new study says the season you're born in can determine your temperament — and one season has a surprising outcome. Video provided by Newsy
Powered by NewsLook.com
Movies Might Desensitize Violence For Parents, Not Just Kids

Movies Might Desensitize Violence For Parents, Not Just Kids

Newsy (Oct. 20, 2014) A study suggests that parents become desensitized to violent movies as well as children, which leads them to allow their kids to view violent films. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins