• Researchers at the Francis Crick Institute and UCL have shown that hundreds of proteins and mRNA molecules are found in the wrong place in nerve cells affected by Motor Neuron Disease (MND), also known as Amyotrophic Lateral Sclerosis (ALS). (news-medical.net)
  • Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a fatal motor neuron disease that causes people to gradually lose control of their muscles. (news-medical.net)
  • Research at The University of Queensland could eventually help develop viable treatments - and ultimately a cure - for motor neuron disease (MND). (news-medical.net)
  • A study suggests that spinal muscular atrophy (SMA), a genetic neuromuscular disease in infants and children, results primarily from motor circuit dysfunction, not motor neuron or muscle cell dysfunction, as is commonly thought. (neurosciencenews.com)
  • Researchers have identified mutations in a number of genes which could be associated with the development of spontaneously occurring cases of ALS, a progressive and fatal neurodegenerative disease in which motor neurons gradually cease to function. (neurosciencenews.com)
  • New research could help find a solution to slowing down the progression of motor neuron disease. (neurosciencenews.com)
  • The research may lead to treatments for motor neuron disease and spinal cord injury. (sci-info-pages.com)
  • On Friday, the motor neuron research group took on the ALS Ice Bucket Challenge to help raise awareness for this rapidly progressive neurodegenerative disease. (evotec.com)
  • Amyotrophic lateral sclerosis ("ALS") - also referred to as motor neuron disease or Lou Gehrig's disease in some part of the United States - is a debilitating disease with varied etiology characterised by rapidly progressive weakness, muscle atrophy and fasciculations, muscle spasticity, difficulty speaking (dysarthria), difficulty swallowing (dysphagia) and difficulty breathing (dyspnea). (evotec.com)
  • The Ice Bucket Challenge is an activity involving dumping a bucket of ice water on someone's head to promote awareness of the disease amyotrophic lateral sclerosis (ALS) and encourage donations to research. (evotec.com)
  • The electrophysiological findings may be explained by degeneration spreading to the lower motor neurons, thus contributing to disease progression. (hspersunite.org.au)
  • Patients with motor neuron disease (MND) are generally free of cognitive impairment, but evidence is growing to support an association between MND and frontal lobe or frontotemporal dementia (FTD). (medscape.com)
  • Worldwide, frontotemporal lobe dementia with motor neuron disease (FTD/MND) is a sporadic condition with an unknown etiology. (medscape.com)
  • Researchers have identified a gene, called matrix metalloproteinase-9 (MMP-9), that appears to play a major role in motor neuron degeneration in amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. (sciencedaily.com)
  • The findings, made in mice, explain why most but not all motor neurons are affected by the disease and identify a potential therapeutic target for this still-incurable neurodegenerative disease. (sciencedaily.com)
  • One of the most striking aspects of ALS is that some motor neurons -- specifically, those that control eye movement and eliminative and sexual functions -- remain relatively unimpaired in the disease," said study leader Christopher E. Henderson, PhD, the Gurewitsch and Vidda Foundation Professor of Rehabilitation and Regenerative Medicine, professor of pathology & cell biology and neuroscience (in neurology), and co-director of Columbia's Motor Neuron Center. (sciencedaily.com)
  • In other words, having MMP-9 is an absolute predictor that a motor neuron will die if the disease strikes, at least in mice. (sciencedaily.com)
  • Avoiding false positive diagnoses of motor neuron disease: lessons from the Scottish Motor Neuron Disease Register. (bmj.com)
  • OBJECTIVES--To describe the frequency and characteristics of those patients initially registered with the Scottish Motor Neuron Disease Register (SMNDR) but who subsequently had a diagnosis other than MND made (false positives), to analyse the features which led to a revised diagnosis, and to draw conclusions which might improve routine neurological practice. (bmj.com)
  • METHODS--The Scottish Motor Neuron Disease Register is a community based, prospective disease register to identify and follow up all incident cases of motor neuron disease in Scotland. (bmj.com)
  • Three University of Queensland researchers have each received $1 million from the FightMND foundation for clinical trials to help fast-track potential treatments for motor neurone disease. (edu.au)
  • UQ Centre for Clinical Research and Queensland Brain Institute's Associate Professor Robert Henderson and QBI's Professor Perry Bartlett , along with UQ School of Biomedical Sciences Associate Professor Trent Woodruff will trial drugs that may protect motor neurons and delay progression of the disease. (edu.au)
  • These translational research grants from FightMND will accelerate the work of UQ's world-class researchers in developing treatments for this devastating disease," Professor Ward said. (edu.au)
  • The purpose of the Foundation is to support research in Motor Neurone Disease (MND), also known as Amyotrophic Lateral Sclerosis (ALS) undertaken by Trinity's MND research group. (rmn.ie)
  • An insightful journey into Motor Neurone Disease (MND) research and care presented by a distinguished panel of experts. (rmn.ie)
  • This study day presents a valuable opportunity for healthcare professionals from diverse sectors to convene, fostering a deeper understanding of Motor Neurone Disease. (rmn.ie)
  • Hardiman is a leading authority on the causes, diagnosis, and treatment of amyotrophic lateral sclerosis (ALS) and other forms of motor neuron disease (MND). (rmn.ie)
  • The launch of precision ALS programme is an important step forward in research to find effective therapies for motor neurone disease. (rmn.ie)
  • Genetically targeted therapy to treat motor neurone disease (MND) could be a turning point for. (researchaether.com)
  • According to a recent study by researchers at the Uniformed Services University of the Health Sciences (USU) 1 , a motor neuron disease medication might also have the potential to help treat posttraumatic stress disorder (PTSD). (psychiatrictimes.com)
  • Background Apart from increasing age and a few specific genetic polymorphisms, the aetiology of Motor Neurone Disease is largely unknown. (bmj.com)
  • The basis of hyper-excitability in upper motor neurons in a model of motor neuron disease. (brainfoundation.org.au)
  • Evidence from human motor neuron disease patients and from animal models of motor neurone disease suggests that both upper and lower motor neurones may generate higher than normal levels of activity, and this hyperactivity may play a role in neuron death in motor neurone disease. (brainfoundation.org.au)
  • In contrast to the extensive research carried out on lower motor neurones in animal models of motor neurone disease, little is known about changes in activity in upper motor neurones in these animal models. (brainfoundation.org.au)
  • My research has already shown that lower motor neurones show hyper-activity from birth in an animal model of this disease, and this hyper-activity is associated with an increase in a specific type of sodium current and with increased levels of several sodium channel genes. (brainfoundation.org.au)
  • The aim of this grant is to develop a novel animal model, in which upper motor neurones are positively identified due to their selective expression of a fluorescent protein marker, and then to test the hypothesis that early hyper-excitability, due to this increased sodium current, is present in upper motor neurones in this novel animal model of motor neurone disease. (brainfoundation.org.au)
  • This work will strengthen the evidence for hyperactivity as a factor in motor neurone disease, and may ultimately lead to novel therapeutic strategies. (brainfoundation.org.au)
  • Prof. Dr Ludo Van Den Bosch is awarded the Generet Prize for Rare Diseases (€ 1,000,000) for his research into motor neuron disease ALS. (kbs-frb.be)
  • The diagnosis of motor neurone disease (MND) has a profound effect on the functioning and well-being of both the patient and their family, with studies describing an increase in carer burden and depression as the disease progresses. (open.ac.uk)
  • These include motor neuron disease and Parkinson's disease. (medicalnewstoday.com)
  • The charity's director of research and development, Dr. Arthur Roach, describes the findings as "particularly encouraging," and that the need for new treatments for the disease is urgent. (medicalnewstoday.com)
  • ALS is the most common motor neuron disease which causes the deterioration of the upper and lower motor neurons. (cdc.gov)
  • Secondary cases occurred among genetically related and through migration of affected persons from villages along unrelated persons in a setting of prolonged intrahousehold the Viliui River in Eastern Siberia, where VE has been en- contact with a patient manifesting the disease. (cdc.gov)
  • Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease (MND). (msdmanuals.com)
  • Background: Huntington's disease (HD) is a motor and cognitive neurodegenerative disorder due to prominent loss of striatal medium spiny neurons (MSNs). (lu.se)
  • The research group studies the underlying pathogenic mechanisms of the neurodegenerative and neuropsychiatric Huntington disease (HD) with the ultimate aim to develop novel effective therapies. (lu.se)
  • Her first original research article is in press titled "Effects of mutant huntingtin in oxytocin neurons on non-motor features of Huntington's disease" in Neuropathology and Applied Neurobiology. (lu.se)
  • ALS, also known as Lou Gehrig disease, is the most common neurodegenerative disease of adult onset involving the motor neuron system. (medscape.com)
  • Combining cell biology and electrophysiology, his work has the potential to create personalized disease models for future research. (lu.se)
  • New research offers clues about the biology of cells in the spinal cord that die off in amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. (news-medical.net)
  • These neurons degenerate in motor neuron diseases, and their damage contributes to loss of motor function in spinal cord injuries. (sci-info-pages.com)
  • Their previous work suggested that cellular pathways involved in processing fat molecules may be a common factor underlying HSP and related motor neuron diseases. (the-scientist.com)
  • ALS is the most common of the five motor neuron diseases. (evotec.com)
  • Motor neuron diseases like amyotrophic lateral sclerosis (ALS) destroy motor neurons. (pluto.bio)
  • Currently, there are no treatments available to fully prevent the progression of motor neuron diseases. (pluto.bio)
  • Researchers around the world are studying motor neuron diseases to better understand how to treat them. (pluto.bio)
  • One challenge currently slowing progress in this field is that there are few reliable in vitro models of motor neuron diseases. (pluto.bio)
  • I developed a system for generating large quantities of mature human motor neurons in vitro that can be used to study all types of motor neuron diseases, as well as potential therapies, in the lab. (pluto.bio)
  • In the human body, these motor neurons innervate the legs, making them a perfect model system to study motor neuron diseases. (pluto.bio)
  • Finally, we explored the method for "external validity" in a dual approach: First, we tested whether the degree of cortical involvement is mirrored by our tool (which we hypothesized given it exclusively targets supratentorial gray matter regions) by contrasting different motor neuron diseases (MND) against each other (study 3). (uni-regensburg.de)
  • The purpose of this research is to get a better picture of who gets ALS or other motor neuron diseases. (cdc.gov)
  • The Vollum Institute is a privately endowed research institute at Oregon Health & Science University dedicated to basic research that will lead to new treatments for neurological and psychiatric diseases. (ohsu.edu)
  • Amyotrophic lateral sclerosis and other motor neuron diseases are characterized by steady, relentless, progressive degeneration of corticospinal tracts, anterior horn cells, bulbar motor nuclei, or a combination. (msdmanuals.com)
  • Researching and developing new treatments for rare diseases is time consuming, complex, and often expensive. (medlineplus.gov)
  • This article describes two rare diseases - spinal muscular atrophy and Duchenne muscular dystrophy - and how NIH supports research and development on gene therapies to treat them. (medlineplus.gov)
  • To further decode the neural mechanism of DS MSN underlying motor skill learning, we developed a custom miniature fluorescent microscope (miniScope) to concurrently record calcium activities of hundreds of striatal neurons longitudinally from mice training on accelerating rotarod. (nih.gov)
  • Locomotion activates PKA through dopamine and adenosine in striatal neurons. (ohsu.edu)
  • Conclusions: The data support the long-term therapeutic potential of ESC-derived human striatal progenitor grafts for the replacement of degenerated striatal neurons in HD and suggest that EE can effectively accelerate the maturation and promote the integration of human striatal cells. (lu.se)
  • Signs of lower motor neuron dysfunction were found in this study of people with HSP and PLS via electrophysiological markers. (hspersunite.org.au)
  • Further, the researchers found that MMP-9 can be detected not just in lumbar 5 neurons, but also in other types of motor neurons affected by ALS. (sciencedaily.com)
  • It may sound funny, but when we set out to do this experiment, we weren't sure which types of motor neurons the protocol would actually produce. (pluto.bio)
  • So, MMP-9 is not only labeling the most vulnerable groups of motor neurons, it is labeling the most vulnerable subtypes within those groups, as well," said Dr. Spiller. (sciencedaily.com)
  • In addition, groups of motor neurons were partly decoupled from their innervated muscle, such that motor neurons innervating the same muscle did not necessarily receive common inputs. (nih.gov)
  • The study supports the theory that movements are produced through the control of small numbers of groups of motor neurons via common inputs and that there is a partial mismatch between these groups of motor neurons and muscle anatomy. (nih.gov)
  • Survival of motor neuron or survival motor neuron (SMN) is a protein that in humans is encoded by the SMN1 and SMN2 genes. (wikipedia.org)
  • They found that TDP-43 depletion in human motor neurons alters RNA transcript levels of some proteins, notably, the microtubule regulator STMN2 . (broadinstitute.org)
  • MND, as the name suggests, is a pure motor disorder without any significant evidence of sensory symptoms, extraocular movement disturbances, bladder and bowel dysfunction, or cognitive impairment. (medscape.com)
  • Progressive dementia with symptoms of executive dysfunction, personality change, and motor weakness leads to severe morbidity. (medscape.com)
  • Signs and symptoms reflect frontal and temporal lobe dysfunction with lower motor neuron-type weakness, muscle atrophy, and fasciculations. (medscape.com)
  • Importantly, individuals with the mutant HD gene suffer from psychiatric symptoms and metabolic dysfunction many years before onset of typical motor symptoms. (lu.se)
  • MND research in Ireland is led by Orla Hardiman, Professor of Neurology at Trinity College Dublin and a world authority on MND and related neurodegeneration. (rmn.ie)
  • Professor Orla Hardiman of Trinity College Dublin has once again been recognised for her remarkable contributions to research into neurodegenerative conditions. (rmn.ie)
  • A research team describes the entire network of brain cells that are connected to specific motor neurons controlling whisker muscles in newborn mice. (neurosciencenews.com)
  • Researchers have found that the insulin-like growth factor 1 (IGF-1) dramatically increases the in vitro growth of corticospinal motor neuron (CSMN) axons - projections that carry nerve impulses to the spinal motor neurons that connect to muscles. (sci-info-pages.com)
  • Movements are reportedly controlled through the combination of synergies that generate specific motor outputs by imposing an activation pattern on a group of muscles. (nih.gov)
  • Specifically, high-density surface electromyography recordings from six lower limb muscles were decomposed into motor neurons spiking activity. (nih.gov)
  • Conversely, some motor neurons from different muscles-including distant muscles-received common inputs. (nih.gov)
  • We decoded the spiking activities of dozens of spinal motor neurons innervating six muscles during a multi-joint task, and we used a purely data-driven method grounded on graph theory to extract networks of motor neurons based on their correlated activity (considered as common input). (nih.gov)
  • When muscles don't receive normal signals from our motor neurons, the muscles atrophy (weaken and shrink) and we lose the ability to do those activities over time. (pluto.bio)
  • Motor neurons send signals to the muscles. (cdc.gov)
  • In Silver syndrome, only specific motor neurons are involved, resulting in the hand and leg muscles being solely affected. (medlineplus.gov)
  • Lower MNDs affect the anterior horn cells or cranial nerve motor nuclei or their efferent axons to the skeletal muscles. (msdmanuals.com)
  • PBP is a progressive degenerative disorder of the motor nuclei in the medulla (specifically involving the glossopharyngeal, vagus, and hypoglossal nerves) that produces atrophy and fasciculations of the lingual muscles, dysarthria, and dysphagia. (medscape.com)
  • It is characterized by pyramidal cell loss in the frontal and temporal lobes and degeneration of motor neurons in the hypoglossal nucleus and spinal motor neurons. (medscape.com)
  • Conclusions: Intrathecal delivery of ALS CSF induces motor weakness and degeneration of motor neurons in the cervical spinal cord by 1 day post injection. (tischms.org)
  • Progressive deficits in retrograde axon transport precede degeneration of motor axons in acrylamide neuropathy. (cdc.gov)
  • Accumulated decrements in retrograde axon transport may be the underlying cause of degeneration of motor axons in acrylamide neuropathy in fowl. (cdc.gov)
  • Jeffrey Macklis, director of the MGH-Harvard Medical School Center for Nervous System Repair, said: "Our findings that IGF-1 specifically enhances both the speed and extent of axon outgrowth of corticospinal motor neurons are the first direct evidence of growth factor control over the differentiation of these neurons. (sci-info-pages.com)
  • Motor neurons, which degenerate and die in ALS, are large cells with one long extension called an axon ― up to a meter long ― that conducts signals from the spinal cord to the muscle fiber. (nih.gov)
  • The team found that motor neurons are distinguished by a set of genes that may enable the large size of the motor neuron cell body and lengthy axon, but also underlie their vulnerability to degeneration. (nih.gov)
  • As a motor neuron matures, it develops an axon, which is used for its main function of transmitting signals long distances across the body. (pluto.bio)
  • Single injection of acrylamide (1.3 mmol/kg, i.p.) inhibited retrograde axon transport of [125I]tetanus toxin in hen sensory and motor axons. (cdc.gov)
  • Deficits were also observed in sensory neurons but were not statistically significant. (cdc.gov)
  • Findings appeared in Neuron . (nih.gov)
  • Research findings indicate that misfolded seipin proteins accumulate in the endoplasmic reticulum. (medlineplus.gov)
  • These findings highlight the need for additional research among caregivers of patients with less advanced cancer or with different illnesses. (bvsalud.org)
  • 2021: Research assistant in Stanley Heinze's research group at Functional zoology, Department of biology, Lund university, Lund, Sweden. (lu.se)
  • The neurons were taken from normal mice. (sciencedaily.com)
  • The same effect on motor neuron function was seen when MMP-9 was inactivated in SOD1 mutant mice using chemical injections or virally mediated gene therapy. (sciencedaily.com)
  • Additional experiments showed that ALS-related genes are also enriched in motor neurons in mice. (nih.gov)
  • Forelimb motor deficits were assessed at 1 day post injection, then mice were perfused for histological analyses of the spinal cord. (tischms.org)
  • Increased death of motor neurons was revealed by a significantly fewer number of ChAT-positive motor neurons and increased activated-caspase3 staining in the cervical spinal cords of ALS CSF-injected mice. (tischms.org)
  • 40 top research centres in 14 countries have joined hands with patient organisations and fundraisers to reach one goal: find effective treatments for ALS. (rmn.ie)
  • Researchers and clinicians at the Brain Health Research Centre are involved with all stages of brain research, from puzzling out the basic mechanisms of how the brain works, to finding treatments that harness the brain's restorative potential and then testing innovative therapies. (otago.ac.nz)
  • Support Tisch MS and our innovative research leading to treatments that improve the lives of patients. (tischms.org)
  • This type of cutting edge research is the best hope of finding better treatments for people with Parkinson's in years, not decades," he says. (medicalnewstoday.com)
  • In a second study, the researchers identified the molecular pathway in SMA that leads to problems with motor function. (neurosciencenews.com)
  • To understand why only some motor neurons are vulnerable to ALS, the researchers used DNA microarray profiling to compare the activity of tens of thousands of genes in neurons that resist ALS (oculomotor neurons/eye movement and Onuf's nuclei/continence) with neurons affected by ALS (lumbar 5 spinal neurons/leg movement). (sciencedaily.com)
  • In a follow-up experiment, the researchers confirmed that the product of MMP-9, MMP-9 protein, is present in ALS-vulnerable motor neurons, but not in ALS-resistant ones. (sciencedaily.com)
  • Taking a closer look at the groups of vulnerable motor neurons, the researchers found differences in MMP-9 expression at the single-cell level. (sciencedaily.com)
  • The researchers are still investigating how MMP-9 affects motor neuron function. (sciencedaily.com)
  • Deputy Vice-Chancellor (Research) Professor Robyn Ward said the grants would support UQ researchers to find potential therapies for patients with MND. (edu.au)
  • A team of researchers supported by the National Institutes of Health found evidence linking motor neurons' large cell size and supporting structure with the genes that underlie their vulnerability to degeneration in ALS. (nih.gov)
  • Research areas Through programs spanning genetics, biology, and therapeutic development, Broad researchers are making discoveries that drive biomedical science forward. (broadinstitute.org)
  • We're honouring all of those researchers in Trinity who've actually turned that research into innovation and these awards, I suppose, are our chance once a year to do that and to acknowledge the great work that they've done," she said. (siliconrepublic.com)
  • Although their cell bodies are located in the brain, CSMN axons extend down to the neurons they control in the spinal cord - extending as far as three feet in adult humans. (sci-info-pages.com)
  • The soma, the round-ish cell body of a motor neuron, is typically about 10 micrometers in humans, which is large enough to see through a microscope. (pluto.bio)
  • In addition to providing insight into the development and circuit formation of this critical population of neurons, these results might lead to the future ability to treat motor neuron disorders and spinal cord injuries. (sci-info-pages.com)
  • Andreas' research focuses on generating brain cells, specifically interneurons, in the lab, which has significant implications for the study of neurodegenerative and neuropsychiatric disorders. (lu.se)
  • The National Institute of Neurological Disorders and Stroke (NINDS) supports research and development on therapies that work at the genetic level to "fix" those problems in children and adults with SMA. (medlineplus.gov)
  • In 110 patients with sporadic adult-onset upper motor neuron syndromes, a novel REEP1 mutation was identified in one patient. (rug.nl)
  • Progeny from this cross with no MMP-9 exhibited an 80-day delay in loss of fast-fatigable motor neuron function and a 25 percent longer lifespan, compared with littermates with two copies of the MMP-9 gene. (sciencedaily.com)
  • To our surprise, the motor neurons we created expressed the same gene (HOXC8) as motor neurons located specifically in the thoracic/lumbar section of the spinal cord. (pluto.bio)
  • Barplot showing HOX gene expression in generated motor neurons. (pluto.bio)
  • There's another gene-based therapy for children under age 2 that uses a safe virus to deliver a new copy of the SMN gene into specific neurons to improve muscle movement. (medlineplus.gov)
  • Data collected by the National ALS Registry are being used to better describe the epidemiology of ALS in the United States and to help facilitate research. (cdc.gov)
  • SMN deficiency, primarily due to mutations in SMN1, results in widespread splicing defects, especially in spinal motor neurons, and is one cause of spinal muscular atrophy. (wikipedia.org)
  • Background: ALS is a progressive neurodegenerative disorder characterized by motor neuron death. (tischms.org)
  • The disorder induces muscle weakness and atrophy throughout the body caused by the degeneration of the upper and lower motor neurons. (evotec.com)
  • Motor neurons are important cells in our body that send signals to our skeletal muscle that allow us to do activities like walk, speak, swallow, and even breathe. (pluto.bio)
  • This accumulation likely damages and kills motor neurons, which leads to muscle weakness and spasticity. (medlineplus.gov)
  • Dorsal striatum (DS) medial spiny neurons(MSN) of direct pathway (expressing D1 receptor, D1-MSN) and indirect pathway (expressing D2 receptor, D2-MSN) are involved in motor skill learning, however, the underlying neural mechanism remains largely unknown. (nih.gov)
  • Deciphering mechanically activated ion channels at the single-channel level in dorsal root ganglion neurons. (ohsu.edu)
  • Read our 2022 Newsletter for an overview of key events and research involving RMN over the last 12 months. (rmn.ie)
  • A loss-of-function mutant, cnd-1(ju29 ), exhibited multiple defects in the ventral cord motor neurons. (biologists.com)
  • Second, the strict correlation between the fate of a motor neuron with respect to its lineage and position in the ventral cord was disrupted, as manifested by the variable expression pattern of motor neuron fate specific markers. (biologists.com)
  • Our data suggest that cnd-1 may specify the identity of ventral cord motor neurons both by maintaining the mitotic competence of their precursors and by modulating the expression of neuronal type-specific determination factors. (biologists.com)
  • We found a number of candidate 'susceptibility' genes -- genes that were expressed only in vulnerable motor neurons. (sciencedaily.com)
  • The electrical activity of upper motor neurones and the levels of expression of sodium channel genes and proteins will then be measured for the first time in these novel animals. (brainfoundation.org.au)
  • To grow motor neurons in vitro in a way that accurately represents how they grow in vivo, we designed a protocol for guiding them through the developmental stages above. (pluto.bio)
  • This thesis encompasses four original research papers which taken together describe and validate an individualized atrophy-assessment tool: first, the general procedure of rating an individual's MRI data, specifically cortical thickness data, to a control population is investigated for sensitivity and specificity using simulations. (uni-regensburg.de)
  • Our hypothesis is that the non-motor features are due to hypothalamic pathology. (lu.se)
  • The present study is an examination of possible subclinical involvement of lower motor neuron (LMN) in patients with primary lateral sclerosis (PLS) and hereditary spastic paraparesis (HSP) electrophysiologically. (hspersunite.org.au)
  • BSCL2 mutations in two Dutch families with overlapping Silver syndrome-distal hereditary motor neuropathy. (medlineplus.gov)
  • The complex, apart from the "proper" survival of motor neuron protein, includes at least six other proteins (gem-associated protein 2, 3, 4, 5, 6 and 7. (wikipedia.org)
  • Fast-fatigable neurons (which are involved in movements like jumping and sprinting and are the first to die in ALS) were found to have the most MMP-9 protein, whereas slow neurons (which control posture and are only partially affected in ALS) had none. (sciencedaily.com)
  • We thought that if we could find out why these neurons have a natural resistance to ALS, we might be able to exploit this property and develop new therapeutic options. (sciencedaily.com)
  • Inactivation of C2 or the closely related C3 neurons with highly specific intersectional driver lines consistently reproduced hyperactive climbing whereas strong or weak artificial depolarization of C2/C3 neurons strongly or mildly decreased climbing frequency. (nature.com)
  • Generated motor neurons are specific to the HOXC8 region of the spinal cord. (pluto.bio)
  • One reason for this is that upper motor neurones are only a fraction of cortical neurones, and it is consequently difficult to positively identify the upper motor neurones in experimental research. (brainfoundation.org.au)
  • Whether this early hyper-excitability also exists in upper motor neurones is unknown. (brainfoundation.org.au)
  • We provide a new neural framework for a deeper understanding of the structure of common inputs to motor neurons. (nih.gov)
  • She said, "My genuine hope is that I, and our research group, have made an impact that will outlast our own professional careers and that we will in the future be able to say that ALS/MND is a chronic, rather than a fatal, condition. (rmn.ie)
  • A better understanding of such motor control circuits could help inform how human brains develop, potentially leading to new ways of restoring movement in people who suffer paralysis from brain injuries, or to the development of better prosthetics for limb replacement. (neurosciencenews.com)
  • KEY POINTS: A central and unresolved question is how spinal motor neurons are controlled to generate movement. (nih.gov)
  • They demonstrated the usefulness of the atlas by looking closely at motor neurons, which provide voluntary movement and motor control. (nih.gov)
  • Third, motor neurons also exhibited defects in terminal differentiation characteristics including axonal morphology and synaptic connectivity. (biologists.com)
  • Ongoing research is needed to discover the cause, treatment and methods of improving quality of life for MND sufferers and their families. (rmn.ie)
  • Research also showed a possible role of SMN in neuronal migration and/or differentiation. (wikipedia.org)
  • She stated that throughout her career, Hardiman has put her patients and their families first, which has been the main driver for her research and contribution to advancing professional practice. (rmn.ie)
  • The research explore the biological reason why different patients with ALS have different trait. (rmn.ie)
  • Once we had produced the cells that we wanted to study, we decided to subject them to hypoxic conditions (lower than normal oxygen levels) because it has been reported that motor neurons of diabetic patients experience hypoxic conditions due to poor blood circulation in the periphery (i.e. the patient's feet). (pluto.bio)
  • Using advanced engineering research to deliver the best care for patients. (kcl.ac.uk)
  • Research released today, funded by the MND Association, has found higher than expected genetic changes in a group of 100 MND patients. (mndassociation.org)
  • The World Health Organization, through its International Clinical Trials Registry Platform, has developed the ICTRP database to provide patients, family members and members of the public current information about clinical research studies. (who.int)