Study gives clues to causes of motor neurone disease

October 10, 2012
Study gives clues to causes of Motor Neurone Disease
The team used neurones derived from embryonic stem cells for the study.

(Medical Xpress)—Scientists at the University of Bath are one step further to understanding the role of one of the proteins that causes the neurodegenerative disorder, Amyotrophic Lateral Sclerosis (ALS), also known as Motor Neurone Disease (MND).

The scientists studied a protein called angiogenin, which is present in the spinal cord and brain that protects neurones from cell death. Mutations in this protein have been found in sufferers of MND and are thought to play a key role in the progression of the condition.

MND triggers , and muscle twitches and spasms. The disease affects around 5000 people in the UK.

The team of and structural biologists have, for the first time, produced images of the 3D structures of 11 mutant versions of angiogenin to see how the mutations changed the structure of the active part of the molecule, damaging its function.

The study, published in the prestigious journal Nature Communications, provides insights into the causes of this disease and related conditions such as Parkinson's Disease.

The team also looked at the effects of the malfunctioning proteins on neurones grown from in the laboratory.

They found that some of the mutations stopped the protein being transported to the , a process that is critical for the protein to function correctly.

The mutations also prevented the cells from producing stress granules, the neurone's natural defence from stress caused by low .

Dr Vasanta Subramanian, Reader in Biology & Biochemistry at the University, said:

"This study is exciting because it's the first time we've directly linked the structure of these faulty proteins with their effects in the cell.

"We've worked alongside Professor Ravi Acharya's group to combine structural knowledge with cell biology to gain new insights into the causes of this devastating disease.

"We hope that the scientific community can use this new knowledge to help design new drugs that will bind selectively to the defective protein to protect the body from its damaging effects."

The findings were welcomed by medical research charity, the (MND) Association, the only national charity in England, Wales and Northern Ireland dedicated to supporting people living with MND while funding and promoting cutting-edge global research to bring about a world free of the disease.

Dr Brian Dickie, Director of Research Development at the charity, said: "The researchers at the University of Bath have skilfully combined aspects of biology, chemistry and physics to answer some fundamental questions on how angiogenin can damage motor neurones. It not only advances our understanding of the disease, but may also give rise to new ideas on treatment development."

Explore further: Stem cell study aids quest for motor neurone disease therapies

More information: www.nature.com/ncomms/journal/ … full/ncomms2126.html

Related Stories

Stem cell study aids quest for motor neurone disease therapies

March 26, 2012
A breakthrough using cutting-edge stem cell research could speed up the discovery of new treatments for motor neurone disease (MND).

Cell study may aid bid for motor neurone therapies

February 28, 2012
The quest for treatments for motor neurone disease, spinal cord injury and strokes could be helped by new research that shows how key cells are produced.

Recommended for you

Molecular hitchhiker on human protein signals tumors to self-destruct

July 24, 2017
Powerful molecules can hitch rides on a plentiful human protein and signal tumors to self-destruct, a team of Vanderbilt University engineers found.

Researchers develop new method to generate human antibodies

July 24, 2017
An international team of scientists has developed a method to rapidly produce specific human antibodies in the laboratory. The technique, which will be described in a paper to be published July 24 in The Journal of Experimental ...

New vaccine production could improve flu shot accuracy

July 24, 2017
A new way of producing the seasonal flu vaccine could speed up the process and provide better protection against infection.

A sodium surprise: Engineers find unexpected result during cardiac research

July 20, 2017
Irregular heartbeat—or arrhythmia—can have sudden and often fatal consequences. A biomedical engineering team at Washington University in St. Louis examining molecular behavior in cardiac tissue recently made a surprising ...

Want to win at sports? Take a cue from these mighty mice

July 20, 2017
As student athletes hit training fields this summer to gain the competitive edge, a new study shows how the experiences of a tiny mouse can put them on the path to winning.

'Smart' robot technology could give stroke rehab a boost

July 19, 2017
Scientists say they have developed a "smart" robotic harness that might make it easier for people to learn to walk again after a stroke or spinal cord injury.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.