Navigation Links
Misplaced molecules: New insights into the causes of dementia
Date:3/1/2013

This press release is available in German.

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 of the USA" (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."


'/>"/>

Contact: Dirk Frger
presse@dzne.de
0049-228-433-02260
Helmholtz Association of German Research Centres
Source:Eurekalert

Related biology news :

1. Study provides insights into plant evolution
2. Life experiences put their stamp on the next generation: New insights from epigenetics
3. Discovery of sexual mating in Candida albicans could provide insights into infections
4. New insights into the borderline personality brain
5. Study offers new insights into the mechanics of muscle fatigue
6. New insights into how immune system fights atherosclerosis
7. Study offers insights into role of muscle weakness in Down syndrome
8. Basketball teams offer insights into building strategic networks
9. Research provides new insights into dogs natural feeding behavior and finds they target a daily dietary intake that is high in fat
10. Insights into a new therapy for a rare form of cystic fibrosis
11. Notre Dame research could provide new insights into tuberculosis and other diseases
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/1/2016)... Favorable Government Initiatives Coupled With ... Identification to Boost Global Biometrics System Market Through 2021  ... report, " Global Biometrics Market By Type, By ... 2011 - 2021", the global biometrics market is projected ... of growing security concerns across various end use sectors ...
(Date:5/20/2016)...  VoiceIt is excited to announce its new ... By working together, VoiceIt and VoicePass will offer ... take slightly different approaches to voice biometrics, collaboration ... usability. Both ... "This marketing and technology partnership allows ...
(Date:5/12/2016)... 2016 WearablesResearch.com , a brand of ... results from the Q1 wave of its quarterly wearables ... consumers, receptivity to a program where they would receive ... insurance company. "We were surprised to see ... Michael LaColla , CEO of Troubadour Research, "primarily because ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... June 23, 2016 , ... Supplyframe, the ... the Supplyframe Design Lab . Located in Pasadena, Calif., the Design Lab’s ... how hardware projects are designed, built and brought to market. , The Design ...
(Date:6/23/2016)... ... June 23, 2016 , ... In a new case report ... detail how a patient who developed lymphedema after being treated for breast cancer benefitted ... change the paradigm for dealing with this debilitating, frequent side effect of cancer treatment. ...
(Date:6/23/2016)... , June 23, 2016  Blueprint Bio, a company ... to the medical community, has closed its Series A ... Nunez . "We have received a commitment ... capital we need to meet our current goals," stated ... us the runway to complete validation on the current ...
(Date:6/23/2016)... YORK , June 23, 2016 ... trading session at 4,833.32, down 0.22%; the Dow Jones Industrial ... S&P 500 closed at 2,085.45, down 0.17%. Stock-Callers.com has initiated ... INFI ), Nektar Therapeutics (NASDAQ: NKTR ), ... Therapeutics Inc. (NASDAQ: BIND ). Learn more about ...
Breaking Biology Technology: