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:3/9/2016)... , March 9, 2016 This BCC ... future states of the RNA Sequencing (RNA Seq) market ... such as instruments, tools and reagents, data analysis, and ... various segments of the RNA-Sequencing market such as RNA-Sequencing ... Identify the main factors affecting each segment and forecast ...
(Date:3/3/2016)... , March 3, 2016  FlexTech, a SEMI ... categories of Innovation, Research & Development, Leadership in Education, ... This is the 9 th year of the ... of companies and individuals from past years . ... on a pre-described set of criteria, by a panel ...
(Date:3/2/2016)... , March 2, 2016 ... of the "Global Biometrics as a ... offering. --> http://www.researchandmarkets.com/research/cmt3hk/global_biometrics ) ... "Global Biometrics as a Service Market 2016-2020" ... --> Research and Markets ( http://www.researchandmarkets.com/research/cmt3hk/global_biometrics ...
Breaking Biology News(10 mins):
(Date:4/29/2016)... ... April 29, 2016 , ... During a two day ... a viable company, CereScan’s CEO, John Kelley, joined other Denver business leaders in ... mentor in the Denver area business community, shared his top fundamental learnings in ...
(Date:4/29/2016)... Italy , April 29, 2016 ... 5.11, the latest update to its industry-leading treatment planning ... shown that Monaco version 5.11 ... can now attain calculation speeds up to four times ... . With the industry,s gold standard ...
(Date:4/28/2016)... Hill, Conn. (PRWEB) , ... April 28, 2016 ... ... of financing and ongoing support for Connecticut's innovative, growing companies, today announced the ... digital health and financial technology (fintech) companies. , “VentureClash looks to ...
(Date:4/28/2016)... ... April 28, 2016 , ... Morris Midwest ... house for regional manufacturers at its Maple Grove, Minnesota technical center, May 11-12. ... Chiron and Trumpf. Almost 20 leading suppliers of tooling, accessories, software and ...
Breaking Biology Technology: