Clues about Motor Neurone Disease

Researchers at The University of Western Australia and Harry Perkins Institute of Medical Research have discovered a new connection between the molecules they study and Motor Neurone Disease (MND) or ALS.

They’ve found a new link between the incurable disease and a tiny cellular structure, known as the paraspeckle, which is found within the nucleus of cells and was first identified by UWA and Perkins researcher Dr Archa Fox 13 years ago.

The paper, “Prion-like domains in RNA binding proteins are essential for building subnuclear paraspeckles”was published today in the eminent Journal of Cell Biology. 

“This is highly significant research,” Dr Fox said. “While it is at a very early stage and is now being tested in human cells within a laboratory setting, it provides hope for people with neurodegenerative diseases and their families.”

The study, in collaboration with researchers in UWA’s School of Chemistry and Biochemistry, and the University of Hokkaido in Japan, shows that while lots of different molecules are involved in MND, many of them come together within paraspeckles.

Co-author, UWA Professor Charlie Bond, said paraspeckles were formed by accumulations of proteins and were important because they helped the cells to cope with stress.

“However, when the same proteins accumulate in the wrong places this causes diseases including MND and other lethal conditions,” Professor Bond said.

“One of the big mysteries in MND is we don’t actually understand what causes it. It mainly affects adults and only a small number of cases have a family history of the disease.

“We don’t know what’s going wrong in the motor neurones that are dying, but now we think there could be a common pathway involving paraspeckles that is faulty in MND patients,” he said.

Other researchers involved in the study include Dr Sven Hennig from the Chemical Genomics Centre at Dortmund in Germany, Professor Swaminathan Iyer from UWA and Professor Tetsuro Hirose from University of Hokkaido in Japan.

(Source: The University of Western Australia, Journal of Cell Biology)