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UCLA stem cells scientists make electrically active motor neurons from iPS cells
February 25, 2009
Discovery demonstrates the feasibility of using iPS-derived motor neurons to model and treat diseases Stem cells scientists at UCLA showed for the first time that human induced pluripotent stem (iPS) cells can be differentiated into electrically active motor neurons, a discovery that may aid in studying and treating neurological disorders. Additionally, the motor neurons derived from the iPS cells appeared to be similar in function and efficiency to those derived from human embryonic stem cells, although further testing needs to be done to confirm that. If the similarities are confirmed, the discovery may open the door for new treatments for neurological disorders using patient-specific cells. The study appears today in the early online edition of the journal Stem Cells. It can be accessed here: http://www.stemcells.com/view/0/index.html."It is clear from the literature that you can make at least immature versions of many different kinds of cells from human iPS cells," said William Lowry, a Broad Stem Cell Research Center scientist, an assistant professor of molecular, cell and developmental biology and senior author of the study. "But there is not a lot of data published describing the generation of fully functional cells from human iPS cells." Lowry and his team used skin fibroblasts and reprogrammed them back into an embryonic state, with the ability to differentiate into any cell type in the human body. They then took those cells and differentiated them into motor neurons. Neurons are the responsive cells in the nervous system that process and transmit information by electrochemical signaling. Motor neurons receive signals from the brain and spinal cord and regulate muscle contraction. The study demonstrates the feasibility of using iPS-derived motor neurons and their progenitors to replace damaged or dead motor neurons in patients with certain disorders. It also opens the possibility of studying motor neuron-related diseases in the laboratory to uncover their causes. Motor neurons are lost in many conditions, including spinal cord injury, Amyotrophic Lateral Sclerosis and Spinal Muscular Atrophy. "A primary objective of human embryonic stem cell and human iPS cell technology is to be able to generate relevant cell types to enable the repair of tissue damage and in vitro modeling of human disease processes," the study states. "Here, we demonstrate the successful generation of electrically active motor neurons from multiple human iPS cell lines and provide evidence that these neurons are molecularly and physiologically indistinguishable from motor neurons derived from human embryonic stem cells." Much may be learned from studying the iPS-derived motor neurons and comparing them to motor neurons derived from patients with neurological disorders to see how they differ. The next step for Lowry and his team is to combine the motor neurons with muscle cells to see if they can stimulate a response. If they do, researchers should be able to see the muscle cells contract. University of California - Los Angeles

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Motor Neuron Disease: A Practical Manual (Oxford Care Manuals)
by Kevin Talbot (Author), Martin Turner (Author), Rachel Marsden (Author), Rachel E. Botell (Author)
There are around 5,000 patients in the UK living with Motor Neuron Disease (amyotrophic lateral sclerosis), and many others shouldering the burden of their care. This fatal neurodegenerative disease leads to progressive muscle weakness and wasting, and there is currently non effective treatment. Managing these patients from their initial presentation, through confirmation of diagnosis and throughout their disease journey is challenging for all healthcare professionals. It requires a multi-disciplinary approach involving neurologists, general practitioners, palliative care physicians, specialist nurses, physiotherapists, occupational therapists, and speech and language therapists. Each member of the team has different priorities to effectively manage the myriad of symptoms and other...
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Motor Neuron Disease (The Facts)
by Kevin Talbot (Author), Rachael Marsden (Author)
Motor neuron disease (MND) is a common but devastating disability that has a profound impact on people's lives. This book provides an easily-accessible guide to the disease for patients with motor neuron disease and their carers. The authors have organised it around a series of the commonest questions asked in their clinic, emphasising the variation in the course of MND and the individual nature of the patient journey through the disease. After an initial description of the symptoms for MND and how neurologists make the diagnosis the authors describe what is known about the causes and how scientists are trying to understand the disease. The book also looks at how a team of specialists can provide support and symptom control for the patient.
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Motor Speech Disorders, 1e
by Frederic L. Darley PhD (Author), Arnold E. Aronson PhD (Author), Joe R. Brown (Author)
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Motor Neuron Disease, An Issue of Physical Medicine and Rehabilitation Clinics, 1e (The Clinics: Orthopedics)
by Greg Carter (Author), Michael Weiss MD (Author)
Half a century ago Lou Gehrig was given his diagnosis: amyotrophic lateral sclerosis. It remains a devastating terminal disease. However, considerable effort has been made in the last decade by some of the premier neurologists, physiatrists, and neuroscientists in the world to elucidate a greater understanding of pathogenic mechanisms, to characterize the disease clinically, and to find greater ways to ease suffering for these patients. In this issue of Physical Medicine and Rehabilitation Clinics of North America, we have brought together some of the world's leading authorities on amyotrophic lateral sclerosis and other motor neuron diseases to emphasize this ongoing research and to explain what the future holds in regard to our further understanding of these diseases.
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Motor Neuron Diseases: Causes, Classification and Treatments (Neurology - Laboratory and Clinical Research Developments)
by Bradley J. Turner (Editor), Julie B. Atkin (Editor)
Motor neuron disease (MND), also commonly known as amyotrophic lateral sclerosis (ALS), is a chronic neurodegenerative disorder of the motor system in adults, characterised by the loss of motor neurons in the cortex, brain stem and spinal cord. This book presents current research from across the globe in the study of the causes, classification and treatments of MND, including membrane trafficking defects as determinants of motor neuron susceptibility and degeneration in ALS; motorneuron specific calcium dysregulation and perturbed cellular calcium homeostasis in ALS; and, stem cells and their application in ALS treatment; excitotoxicity and selective motor neuron degeneration and therapeutic invervention and assistive technology treatments.
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Motor Neuron Disease Research Progress
by Raffaele L. Mancini (Editor)
The motor neuron diseases (or motor neuron diseases) (MND) are a group of progressive neurological disorders that destroy motor neurons, the cells that control voluntary muscle activity such as speaking, walking, breathing, and swallowing. Neurological examination presents specific signs associated with upper and lower motor neuron degeneration. Signs of upper motor neuron damage include spasticity, brisk reflexes and the Babinski sign. Signs of lower motor neuron damage include weakness and muscle atrophy. Every muscle group in the body requires both upper and lower motor neurons to function. It is a common misconception that 'upper' motor neurons control the arms, while 'lower' motor neurons control the legs. The signs described above can occur in any muscle group, including the arms,...
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Waging a War against Motor Neuron Disease: On the Frontier of Translational Research for Lou Gehrig's Disease
by Page Jones (Author)
Despite the great strides that have been made in research for Lou Gehrig?s Disease in the twentieth century, effective treatments are sorely lacking. This is a devastating neurodegenerative disorder that progresses rapidly to paralysis and death. What will we do to address treatment options? Waging a War against Motor Neuron Disease describes the development of a cell model and biochemical assays that may lead to the discovery of mechanisms of ALS. This original work provides groundbreaking evidence that proteomics analysis in spinal cord extracts of ALS mice may yield new protein targets and suggests that therapeutic approaches can be derived from developments in the laboratory. This book describes translational research that is leading to promising...
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Clinical Neurophysiology of Motor Neuron Diseases: Handbook of Clinical Neurophysiology
by Andrew Eisen (Author)
The volume summarizes the most recent knowledge in the field of motor system (upper and lower motor neuron) physiology and pathophysiology as related to diseases of this system. It covers the various aspects of motor system disorders from the motor cortex to the spinal anterior horn cell. Emphasis is placed on clinical disorders as they affect the human motor system and includes critical discussion of advances in techniques for diagnosing and understanding the pathophysiology of disorders affecting the motor system, describing approaches to characterization of disease type, location, severity and prognosis. There is discussion of the specificity and sensitivity of methodological techniques used in diagnosis, with comparison among the various methods.
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Upper Motor Neuron Functions and Dysfunctions
by John Eccles (Author), Milan R. Dimitrijevic (Author)
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Upper Motor Neurone Syndrome and Spasticity: Clinical Management and Neurophysiology (Cambridge Medicine)
by Michael P. Barnes (Editor), Garth R. Johnson (Editor)
Spasticity is a disabling problem for many adults and children with a variety of neurological disorders such as multiple sclerosis, stroke, cerebral palsy and traumatic brain injury. A practical guide for clinicians involved in the management of spasticity, this book covers all aspects of upper motor neurone syndrome from basic neurophysiology and measurement techniques to practical therapy and the use of orthoses. Surgical techniques are also covered, as well as the particular problems of management of spasticity in childhood. In the second edition of this key text, all chapters have been thoroughly updated, with additional coverage of new techniques and new drugs and therapies, whilst continuing the format that has made the first edition the core text in its field. This guide will be...
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