Protein linked to MND progression

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A NEW Motor Neuron Disease (MND) test enables doctors to measure the disease’s progression in patients.

The test was developed by researchers from Flinders University in South Australia in collaboration with the University of Miami and has the potential to lead to better treatments and a cure for the neuro-degenerative disease.

The urine test measures the levels of a key protein found in people with MND or Amyotrophic Lateral Sclerosis (ALS).

As the disease continues to develop in patients, the levels of the protein continue to increase.

Research supervisor from Flinders University Mary-Louise Rogers said the technique was not a diagnostic tool but could be used to study the effectiveness of current treatments.

“To date there has been no marker that has been able to measure disease progression,” she said.

“What is being used is a test where people are asked a bunch of questions by neurologists who take down their answers and add up a score.

“This urine test is simple and could improve treatment for MND by showing us which drugs or methods are working best.”

As nerves get thicker, a protein known as protein75ECD is shed off of motor neurons and released into the blood stream, ending up in the urine.

Previous research in mice linked protein75ECD levels and MND progression.

Researchers later identified higher levels of the protein in 31 people with MND over a three-year period.

The patients were in a number of American and Australian states and the United States, with testing conducted every few months.

There is currently no cure for MND (or ALS), which causes the motor neurons or nerve cells that control muscle movements to slowly die.

MND affects about six people per 100,000.

It is more often found in the 40-to-70-year age group with about 140,000 new cases diagnosed worldwide each year according to the International Alliance of ALS/MND Associations.

ALS, as it is referred to in North America, is also known as Lou Gehrig’s disease, after the baseball great and former New York Yankee who was diagnosed with the illness in 1939.

Other notable individuals with the disease include theoretical physicist and cosmologist Stephen Hawking, Toto bassist Mike Porcaro and Sesame Street creator Jon Stone.

“I’ve been working with researchers in the US and we are putting all our effort into getting it ready for a clinical trial,” Dr Rogers said.

“We hope to do this in the next two to three years.”

Flinders University’s Centre for Neuroscience researcher Stephanie Shepheard and her supervisor Dr Mary-Louise Rogers conducted the comparative study of the testing system on MND and non-MND patients in South Australia over the past six years.

Collaborators and co-authors in the latest paper include leading Flinders Medical Centre MND Clinic researcher and clinician and University of Miami Miller School of Medicine Department of Neurology Professor Michael Benatar, who has trialled the biomarker test on MND patients in the US.

The study was supported by the US National Institutes of Health Rare Diseases Clinical Research Network, Motor Neurone Disease Research Institute of Australia, Flinders University Centre for Neuroscience and Flinders Foundation, ALS Association, Muscular Dystrophy Association, ALS Recovery Fund and Australian Rotary Health (Neville and Jeanne York Family Scholarship).

The paper Urinary p75ECD: A prognostic, disease progression, and pharmacodynamic biomarker in ALS has been published in Neurology.

South Australia’s capital Adelaide has three long-standing public universities, Flinders University, University of South Australia and the University of Adelaide, each of which are consistently rated highly in the international higher education rankings.