Insight into Motor Neuron Disease: Stem Cell Discovery

A discovery using stem cells from a patient with motor neuron disease could help research into treatments for the condition. The study used a patient's skin cells to create motor neurons -- nerve cells that control muscle activity -- and the cells that support them called astrocytes.

They found that a protein expressed by abnormalities in a gene linked to motor neuron disease, which is called TDP-43, caused the astrocytes to die.The study, led by the University of Edinburgh and funded by the Motor Neurone Disease Association, provides fresh insight into the mechanisms involved in the disease.


Although TDP-43 mutations are a rare cause of motor neuron disease (MND), scientists are especially interested in the gene because in the vast majority of MND patients, TDP-43 protein (made by the TDP-43 gene) forms pathological clumps inside motor neurons.


Motor neurons die in MND leading to paralysis and early death.


This study shows for the first time that abnormal TDP-43 protein causes death of astrocytes. The researchers, however, found that the damaged astrocytes were not directly toxic to motor neurons.


Better understanding the role of astrocytes could help to inform research into treatments for MND.


Professor Siddharthan Chandran, of the University of Edinburgh, said: "Motor neuron disease is a devastating and ultimately fatal condition, for which there is no cure or effective treatment. It is not just a question of looking solely at motor neurons, but also the cells that surround them, to understand why motor neurons die. Our aim is to find ways to slow down progression of this devastating disease and ultimately develop a cure."


These findings, published in the journal Proceedings of the National Academy of Sciences., are significant as they show that different mechanisms are at work in different types of MND.


The research, led by the University of Edinburgh's Euan MacDonald Centre for Motor Neurone Research, was carried out in collaboration with King's College, London, Columbia University in New York, the University of California and the Gladstone Institutes in San Francisco.


Dr Brian Dickie, the MND Association's Director of Research Development, said: "From a therapeutic perspective this finding is important because it means that specific treatments targeted at astrocytes may only be relevant and effective, in specific subsets of patients who will have to be carefully selected for drug trials."

Researches towards Motor Neuron Diseases

A broad range of researches are being conducted, aiming to discover the cause of motor neuron diseases, find better treatments, and, ultimately, prevent and cure the disease. We have made a lot of achievements and progress in motor neuron diseases studies.

Various motor neuron diseases animal models are being used to study disease pathology and identify chemical and molecular processes involved in cellular degeneration, thus finding better treatment for the disease. Research options fall largely into three categories: drugs, gene therapy, and stem cells.

• The drug of antibiotic ceftriaxone has been shown to protect nerves by reducing glutamate toxicity—believed by many scientists to play a critical role in the development of ALS, the most common type of motor neuron diseases—in a mouse model of the disease. One study found that cellular ability to manage glutamate can alter the course of ALS. It is a promising drug for motor neuron diseases, especially for ALS.

• The novel compound dexpramipexole has shown neuroprotective properties in multiple preclinical studies of ALS, and it may work by increasing the efficiency of mitochondria, which is the energy-producing portion of the body’s cells. Mitochondria in the motor neurons undergo significant stress in ALS patients. The compound is currently being tested in an industry-sponsored multi-center clinical trial.

• Several early-stage clinical trials are testing the safety and feasibility of novel treatment strategies for motor neuron diseases. These include cell-based approaches such as the transplantation of neural precursor cells into the spinal cord of the patients, and the infusion of so-called “anti-sense” compounds into the fluid that surrounds the spinal cord and brain to block production of toxic SOD1 protein in ALS patients who carry SOD1 mutations.

Clinical trials are testing whether different drugs or interventions are safe and effective in slowing the progression of motor neuron diseases.

The achievements and progress made in motor neuron diseases researches enable us to have a better understanding of the disease and provide better treatments. You are all welcome to contact us if you have any questions about the disease.

A specific case of MOTOR NEURON DISEASE from AUSTRALIA

Name: Mr. Adam

Gender: Male

Age: 54 years old

Country: Australia

Diagnose: Motor Neuron Disease

Admission Datez: 2014-04-02

Days Admitted to the Hospital: 15 days

Before stem cell treatment: He suffered from weakness of the lower limbs 1.5 years ago, from left leg to right leg. It was accompany with muscles jumping and pain. The patient suffered from slight muscle atrophy and the disease progressed gradually. In September of 2013 he was diagnosed with motor neuron disease. In November of 2013 his lower limbs were almost couldn’t be moved. He received treatment in local hospital, but the effect was not good.

His disease was aggravated in 1 year, he and his family were so eager to look for a good treatment for such disease; they searched so many hospitals that can treat it, finally he chose us. From January to March of 2014, his disease aggravate very fast, he couldn’t lift his upper limbs either. The main symptoms before treatment there was obvious muscle atrophy in four limbs. There is almost no strength in his legs. He couldn’t move himself, couldn’t turn over, couldn’t lift four limbs, couldn’t stand, almost everything need family support.

From the onset of disease, the patient had normal spirit, diet and sleep. His had normal defecation. His weight has increased than before, much shrink appears in his body especially in legs and feet.

Stem cell treatment :
Mr. Adams received all relevant examinations. The patient received neural repair treatment and stem cell activating treatment. He received treatment to repair and improve nerves and also to nourish the neurons. This was combined with physical rehabilitation training.

Post-Stem cell treatment:
The patient’s spirit is stable. The diet and sleep is good. The muscle strength has increased slightly and the shrink has decreased. He can turn over with little support. He has got more strength to move wheelchair. And he could lift his arm much more than before. The grasp power of hands has increased 1 level. The muscle strength of the left lower limb has increased to 1 level; the muscle strength of the right lower limb has increased to 1+ level. The whole feeling is better than before. Now he is still insist to do exercise at home, there will be more improvements in the near future.

For more treatment cases please click on http://www.stemcellshealthcare.com/neural-repair-treatment/treatment-cases

We are here Fight against Motor Neuron Disease

The following passage is from “Motor Neuron Together We Stand”, which is really good for sharing to more people.

A：How do we feel when diagnosed with Motor Neuron Disease and the words this is terminal ie you are facing death?

Does this feeling alter if diagnosed in youth, middle age or old age? I don't know as my outlook upon diagnosis was that my youngest child was only 9 and had no Dad...Others I know are in a similar position, even still being pregnant on the day of diagnosis!

How do they cope with Motor Neuron Disease and terminal?

My outlook on being terminally ill alters as this disease progresses. I thought in 2011 (my diagnosis year) nah it won't kill me. Last year I thought I have 20 years to live. This year I think I have a year left on earth. This alteration in attitude does not mean I feel lost and will die sooner, it means I am accepting the terminality.

On top of that I see Stephen the 19year Cancer hero laugh and make the most of life till he died this week. What an example to our terminal existence. We are all terminal and we all have to face death one day........but till that day is here remembered to smile.

FYI: Motor neuron disease is a progressive disease. Some with MND progress slowly, however, some progress fast. Based on current technology, it still a mystery for us to know the causes for MND, how to cure the patient with MND. Even the stem cell transplantation treatment is just to improve some serious condition of the patient for a period. But if the patient is not taken any treatment for the control of the disease, the disease will progress and the patient will feel it gradually.

Motor neurone disease took my love

Katrina Jeffery of Tuckurimba recently lost her husband Chris Jeffery to motor neurone disease.

MAY is Motor Neurone Disease Awareness month and Katrina Jeffery, of Tuckurimba, knows all too well how important awareness of this disease is. Her husband, Chris Jeffery, died two weeks ago from a fight with motor neurone disease.He was just 55, and had been diagnosed in May 2013 after his speech began to slur and the husband had difficulty swallowing - the fact that was finally discovered to be the bulbar onset of the disease - ahead of the rest of his body began slowing. A rare disease without any cure, motor neuron disease attacks the nerves that run motor function, and soon muscles start to waste away. By the end of his life, Mrs Jeffery said, he had no speech at all - a tough feat for someone who had worked in hospitality his whole life. Mr Jeffery, who was well known for running the Star Court Cafe and achieving run the New Italy complex in previous years, was one of just four known sufferers of the disease in Lismore.Throughout his battle, he maintained the most amazing spirit. "Even though he couldn't really express it most of the time, he always had the cheeky grin and light in his eyes that everyone knew," Mrs Jeffery said. "It was really obvious he still had that spirit within him. He fought every single day. He just wasn't about to take anything lying down." Mr Jeffery passed away at home in the company of his wife in the late hours of Easter Monday.They'd been through hell, she said, but to be able to soothe him both at home and give him the peace he needed to pass on meant the world to her. "I have a lot of comfort that he could just pass peacefully; because (the disease) was the cruellest thing he'd ever been through." Mrs Jeffery gave a huge thank you to local physician Dr Robert Lodge and MND NSW, which donated more than $50,000 worth of equipment to the couple. The earlier the treatment is, the best result or control over the disease will achieve.


Tips: Stereotactic Nerve Repair Treatments
Stereotactic nerve repair treatments for the central nervous system are mainly present in three aspects:


1.Replacement therapy: transplant or activate stem cells in the body, make them differentiating into neurons and glial cells. The cells combines with the original nerve cells and form a new neural network, making it function properly.

2.Neural stem cells as genophore: carry target gene and transplant as positioned in order to make cells renewal and treat gene.

3.Activating growth factors and cytokines: induce neural stem cells to repair themselves.