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:3/30/2017)... 30, 2017 Trends, opportunities and forecast in ... by technology (fingerprint, AFIS, iris recognition, facial recognition, hand ... by end use industry (government and law enforcement, commercial ... banking, and others), and by region ( North ... Asia Pacific , and the Rest of ...
(Date:3/27/2017)... 27, 2017  Catholic Health Services (CHS) has ... Society (HIMSS) Analytics for achieving Stage 6 on ... . In addition, CHS previously earned a place ... an electronic medical record (EMR). "HIMSS ... of EMR usage in an outpatient setting.  This ...
(Date:3/23/2017)... -- The report "Gesture Recognition and Touchless Sensing Market by Technology ... Forecast to 2022", published by MarketsandMarkets, the market is expected to be worth ... and 2022. Continue Reading ... ... ...
Breaking Biology News(10 mins):
(Date:10/11/2017)... ... October 11, 2017 , ... ... it will be hosting a Webinar titled, “Pathology is going digital. Is your ... on digital pathology adoption best practices and how Proscia improves lab economics and ...
(Date:10/11/2017)... (PRWEB) , ... October 11, 2017 , ... ... compared the implantation and pregnancy rates in frozen and fresh in vitro ... of progesterone and maternal age to IVF success. , After comparing the results ...
(Date:10/10/2017)... ... October 10, 2017 , ... For the ... won a US2020 STEM Mentoring Award. Representatives of the FirstHand program travelled to ... Experience from US2020. , US2020’s mission is to change the trajectory of STEM ...
(Date:10/10/2017)... SANTA BARBARA, CALIFORNIA (PRWEB) , ... October 10, ... ... risk management, technological innovation and business process optimization firm for the life sciences ... the BoxWorks conference in San Francisco. , The presentation, “Automating GxP ...
Breaking Biology Technology: