Research providing clues about Motor Neurone Disease

A senior researcher at the Harry Perkins Institute of Medical Research has found that the molecules she studies in her work on cancer are also involved in Motor Neurone Disease, or ALS. Dr Archa Fox

Dr Archa Fox is a WA Researcher who discovered a tiny cellular structure within the nucleus of cells 13 years ago. The structure is now known as the paraspeckle.

“This is significant research into neurodegenerative diseases which are currently incurable, particularly MND,” Dr Fox says.

“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.”

The research, published in the eminent Journal of Cell Biology, shows that while lots of different molecules are involved in MND, many of them come together within paraspeckles.

Paraspeckles are formed by accumulations of proteins and are important in human cells because they help 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.

While the research is at a very early stage and being tested in human cells within a laboratory setting, it provides hope for people with neurodegenerative diseases and their families.

The paper, ‘Prion-like domains in RNA binding proteins are essential for building subnuclear paraspeckles’ is in the Journal of Cell Biology.

About Paraspeckles: These tiny structures are found in the nucleus of each human cell. While more still needs to be understood about their function, they are known to be important for cell health in stressful conditions.

About MND: Motor Neurone Disease or Amyotrophic Lateral Sclerosis, has recently become better known due to the Ice Bucket Challenge. For 90 to 95% of cases the cause is not known. About 5-10% of cases are inherited from a person’s parents. It results in the death of neurons that control muscles.

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