Articles tagged with Motor Neurons
Researchers successfully reproduced key ALS symptoms in zebrafish using optogenetic TDP-43, a human protein that forms aggregates upon blue light exposure. The study reveals motor neurons may be damaged before TDP-43 aggregation, suggesting new avenues for ALS treatment.
Researchers at Karolinska Institutet have discovered a gene, Synaptotagmin 13, that protects against motor neuron degeneration in ALS and SMA. Gene therapy was shown to increase life expectancy in animal models.
Astrocytes, a type of cell that supports motor neurons, play an important protective role in the early-stages of sporadic motor neuron disease. When close to motor neurons, these cells help rescue them from misfolded protein TDP-43.
A new study identified Gemin3 as a molecular 'bridge' between genes causing amyotrophic lateral sclerosis (ALS), a neurodegenerative disease. The research holds promise for developing treatments effective for a broad range of ALS patients.
Researchers developed a novel gene-silencing approach to deliver a treatment vector to adult ALS mice, preventing disease onset and blocking progression in animals with existing symptoms. The method uses an adeno-associated virus to deliver shRNA, effectively silencing the SOD1 gene and protecting motor neurons.
A study published in the Journal of Neuromuscular Diseases found Nusinersen treatment improved motor and respiratory functions in adults with longstanding SMA3. Functional testing showed significant improvements on the 6-Minute-Walk-Test, with patients experiencing a mean improvement of 8.25 meters.
AcuraStem has secured a $3 million Phase II funding for its AS-1 Program, a lead small molecule program targeting amyotrophic lateral sclerosis (ALS). The award allows the company to accelerate the AS-1 program and expand its iNeuroRx technology platform.
Researchers at Oregon State University have made an important advance in understanding why certain cells in the nervous system are prone to breaking down and dying, which is what happens in patients with ALS. The study suggests that a protein chaperone called heat shock protein 90 may be critical to neuron survival.
Researchers at Tohoku University have identified a new pathological mediator of amyotrophic lateral sclerosis (ALS) that could lead to further understanding of the disease's molecular breakdown. The study found that a mutated version of FUS gene causes toxic gain of function, leading to axonal branching and degeneration.
Northwestern University scientists have received a $3.1 million grant to investigate drug therapies for ALS, targeting protein aggregation and upper motor neurons. Promising early results suggest compounds may have broader applications for neurodegeneration.
A new study led by Trinity College Dublin researchers has found that genetics account for about 52% of the risk of developing motor neuron disease. The study involved 1117 people diagnosed with MND and found that those who carried an abnormal copy of the C9orf72 gene were more likely to have inherited it from their mothers.
Researchers have identified unique populations of neurons and associated cells in the spinal cords of patients with ALS, which could serve as useful biomarkers for earlier diagnosis. The study found different types of motor neurons and microglia present in less affected regions of patients with focal-onset ALS.
Scientists at the University of Warwick have discovered a new process that activates the fastest molecular motor in neurons, paving the way for new treatments. The research focuses on KIF1C, a tiny protein-based molecular motor that converts chemical energy into mechanical energy to transport cargoes along microtubule tracks.
Researchers found that protein clumps in ALS neurons can be triggered by cellular stress and may provide a potential target for new therapies. Chemical compounds have been identified to prevent this stress-induced accumulation, offering a promising starting point for treating the disease.
Researchers at Oregon State University have discovered a copper compound that improves the condition of mice with damaged motor neurons, potentially slowing ALS progression. The study builds on previous findings and suggests a broader neuroprotective role for copper-ATSM beyond mutant SOD models of ALS.
Increasing Klotho levels improves neurological deficits and prolongs life span in an experimental ALS model. Brain immune cells also play a crucial role in protecting the brain against inflammation and motor neuron loss.
Scientists have identified specific cellular features that enable certain motor neurons to resist ALS, such as the ability to discard damaged proteins and withstand cellular stress. The findings offer a breakthrough in understanding neurodegeneration and potentially developing treatments to make all neurons ALS-resistant.
Researchers at Karolinska Institutet developed a stem cell-based model to study neuron resilience in ALS. The model identified genes contributing to the resilience of oculomotor neurons, which can withstand the disease.
Researchers found that neurons in the motor cortex divide into externally focused and internally focused groups, with externally focused neurons controlling different body parts and internally focused neurons communicating only within the group. The study's findings have implications for brain disorders such as Rett Syndrome, which may...
Researchers found that transplanting human bone marrow-derived endothelial progenitor cells into mice with ALS-like symptoms improved motor function and slowed disease progression. The treatment repaired damage to the blood-spinal cord barrier, leading to increased motor neuron survival and reduced capillary leakage.
Researchers at the Salk Institute have identified a gene mutation that causes motor axon misrouting, leading to improper connection with muscles. The study reveals how p190 acts as a blinder to guide axons outside of the spinal cord, shedding light on the intricate mechanisms of cell signaling and development.
Scientists at Harvard University have identified a potential biomarker and drug target for amyotrophic lateral sclerosis (ALS), a neurological disease that is difficult to diagnose and treat. The gene STMN2 was found to be involved in the loss of motor neurons, which leads to progressive paralysis.
A phase 2 trial found that an oral spray containing THC-CBD reduced spasticity in adults with motor neuron disease, improving muscle tone and pain levels. The treatment was well-tolerated and showed promise as a potential option for managing symptoms.
Researchers at Karolinska Institutet have devised a new method called Axon-seq to study axons and better understand the pathological development of ALS. The method reveals significant differences in RNA profiles between healthy cells and those with mutated SOD1 genes, which causes ALS.
Researchers discovered that neurons in the motor cortex modify their activity as we reach and grasp for objects, allowing groups of neurons to work together to perform precise movements. This finding has significant implications for neuro-prosthetics and brain-computer interfaces.
Researchers have made a significant breakthrough in understanding the mechanisms of amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disorder. The study found that ezogabine treatment can reduce motor neuron excitability in ALS patients, suggesting a potential new therapy for the disease.
Researchers have developed a rodent model that mimics human swallowing problems in ALS, allowing for targeted study of preserving and restoring swallowing function. The research could one day lead to new treatments to slow the disease and improve quality of life for individuals with ALS.
Researchers found that mutations in specific genes also attack sensory neurons, leading to new insights for preventing or reversing ALS. Sensory neurons' axons exhibit similar pathological changes as motor neurons, making them a potential target for discovering and testing molecules to treat ALS.
A new study by UMass Medical School researchers successfully delivers RNAi-based gene therapy to silence SOD1 protein mutations linked to ALS without adverse effects. The therapy, delivered via a viral vector, achieved silencing of over 90% in some motor neurons, suggesting a safe and potentially one-time treatment for humans.
Cuttlefish control camouflage by directly acting on skin cells called chromatophores, producing local changes in contrast. Through statistical analysis of chromatophore output, researchers inferred motor neuron activity and higher levels of control, peering into the brain's camouflage system.
Researchers at Max Planck Institute for Brain Research and Frankfurt Institute for Advanced Studies developed techniques to reveal the cuttlefish brain's control network. By analyzing skin pattern dynamics, they inferred motor neuron activity and predicted higher-level control structures, providing insights into biological camouflage.
A team of MIT engineers created a microfluidic chip with 3-D tissue model of the interface between motor neurons and muscle fibers, replicating the effects of ALS. The researchers tested two drugs in clinical trials and found that giving both restored most of the lost muscle strength.
Researchers used muscle-on-a-chip technology to model ALS, revealing a promising treatment combination that may improve symptoms. The study found that combining two neuroprotective molecules can efficiently cross the blood-brain barrier and recover muscle contraction and neuronal survival.
Researchers have discovered that two types of motor neurons die in ALS patients through distinct mechanisms, potentially leading to the development of more targeted treatments. The study used worm models to investigate the degeneration of spinal and brain neurons in ALS, revealing new insights into the complex nature of the disease.
Researchers study how male fruit flies generate their courtship song using cutting-edge imaging techniques and genetic tools. They discover that the song serves as an honest signal of a male's fitness, with flaws indicating neuromuscular deficits, which can affect flying abilities.
Researchers discovered a link between the protocadherin 17 gene and abnormal brain-eye muscle connections in zebrafish, which may contribute to strabismus. The study suggests that Pcdh17 protein plays a crucial role in positioning neurons correctly in the brain and extending axons to target muscles.
Researchers at Vanderbilt University have identified a key enzyme that drives neuron death in neurodegenerative diseases, including Alzheimer's and ALS. The enzyme histone deacetylase 1 (HDAC1) modifies a molecular motor that triggers signaling agents to travel down the axon, killing the neuron.
Professor Maurizio Pellecchia has received $2.5 million in funding from the National Academy of Sciences and the National Institute of Neurological Disorders and Stroke to study the EphA3 receptor's role in cancer and ALS. His research aims to develop therapeutic agents that target this receptor, which is overexpressed in cancer cells.
A team of clinical neurologists, molecular biologists, and computer scientists have uncovered the earliest events in motor neuron disease using stem cell technology and RNA sequencing data. They discovered that a protein called SFPQ is lost from the nucleus in diseased motor neurons, leading to their death.
A recent study published in Nature Communications has found that ebselen can correct many of the toxic characteristics of a protein causing some cases of hereditary motor neurone disease (MND). The drug-molecule can restore important steps in the SOD1 assembly process, potentially preventing neuronal cell death.
Researchers at Cedars-Sinai have discovered a new way to treat amyotrophic lateral sclerosis (ALS) by transplanting altered neural cells into the brain. Laboratory rats that received the transplants lived 8 percent longer and were free of paralysis 10 percent longer than untreated animals.
A deficiency of TRIF, a key innate immune adaptor, significantly shortens the survival time of ALS mice by allowing the accumulation of toxic reactive oxygens. This study reveals a new role for innate immunity in ALS pathomechanism and provides a clue to develop a therapeutic approach for protecting motor neurons.
Researchers have found that the human brain's tiny blood vessels can trigger the growth of spinal motor neurons, which control muscles, during early development. This discovery could provide insights into diseases such as amyotrophic lateral sclerosis (ALS) and other neurodegenerative disorders.
New research reveals that CD4+Foxp3+ regulatory T-cells (Tregs) are closely linked to ALS progression, with patients having higher numbers of Tregs experiencing slower disease progression. This discovery provides a promising approach for developing new therapies to treat ALS.
A recent study published in PNAS found that disrupted mRNA transport into axons leads to impaired nerve function and cell death, exacerbating conditions like ALS and spinal muscular atrophy. The researchers identified a noncoding RNA, 7SK, as playing a crucial role in these transport complexes.
Changes in neuron size have been linked to the progression of motor neurone disease, with vulnerable neurons increasing in size before symptoms appear. This could lead to new strategies for slowing or halting nerve cell death and improving treatment options.
A Salk Institute study has identified distinct molecular profiles in V2a neurons that control arm and leg movements, shedding light on neural regulation of motor control. The findings could lead to personalized stem-cell-based treatments for repairing spinal cord injuries.
A new study found that antibodies stimulate a molecule in muscle to keep nerves attached, preserving neuromuscular synapses and slowing disease progression in a mouse model of aggressive ALS. The treatment extended the lifespan of ALS mice and improved function of critical breathing muscles.
Researchers discovered that certain species of fish, like the little skate, can walk using a neural and genetic developmental program identical to higher vertebrates. The study found conserved genes and neuronal subtypes essential for limb control in these fin-based movement systems.
Researchers discovered that a mutation in the C9ORF72 gene causes toxicity in nerve cells, leading to ALS and FTD. By reprogramming blood cells into motor nerve cells, they found that adding supplemental protein could stop degeneration, highlighting potential new drug targets.
Researchers at USC discovered a cellular mechanism that limits the number of 'cellular janitors' in the nervous system, leading to increased risk for ALS and frontotemporal dementia. The study found that a mutation in the C9ORF72 gene causes toxicity in nerve cells, resulting in cell death and degeneration.
Researchers at UCLA have uncovered a gene network that promotes the formation of spinal motor neurons in chicken and mouse embryos. The study sheds light on how embryonic development is orchestrated for motor neuron formation, with implications for stem cell-based therapies to repair or study neurodegenerative diseases.
Researchers uncover new insights into how stem cells transform into brain cells controlling leg movements in fruit flies, with implications for understanding comparable systems in humans. The study finds that two critical cell types, born from the same stem cell, facilitate the construction of a mature motor system.
Researchers at Hokkaido University have shed light on the molecular mechanisms behind ALS by studying the depletion of protein TDP-43. The study found that TDP-43 binds to U6 snRNA, stabilizing it, which leads to its degradation and eventual cell death. This discovery may lead to ways to slow or stop neuronal cell death in ALS patients.
Researchers successfully used CRISPR-Cas9 gene editing to disable a defective gene causing amyotrophic lateral sclerosis (ALS) in mice, extending their lifespan by 25% and delaying the onset of muscle-wasting symptoms. The therapy targeted motor neurons, allowing for rescue of these critical cells and enabling improved muscle function.
A University of Missouri researcher has identified a potential target for therapeutics in treating Lou Gehrig's disease (ALS), which may also help recover patients from strokes and other disorders. The study found that an enzyme called NAMPT plays a crucial role in ALS pathogenesis.
Scientists discover dysfunctional autophagy plays a central role in motor neuron diseases, characterized by muscle atrophy and loss. The PLEKHG5 gene controls the degradation of synaptic vesicles, and its dysfunction leads to aggregation and motor neuron disorder progression.
A study by researchers at the University of Basel and FMI identified specific neuron types in the brainstem that regulate high-speed locomotion. These neurons are intermingled with others that can elicit immediate stopping, and their activation can induce full body locomotion.
A team of researchers used stem cell technology to generate motor neurons from ALS patients with FUS mutations, revealing axonal transport defects. Genetic correction and pharmacological inhibition of HDAC6 restored axonal transport, suggesting a potential therapeutic approach for ALS.
Researchers discovered that autophagy, a cellular 'clean-up process', initially slows ALS disease progression but later accelerates its deadly spread through the spinal cord. The study provides new insights into the complex mechanisms of ALS and lays the groundwork for therapies that could eventually prevent its onset.