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