Articles tagged with Amyotrophic Lateral Sclerosis
FutureNeuro, a leading SFI Research Centre, is expanding its research programme with a focus on diagnostics, therapeutics, and digital health. The centre aims to develop precision diagnostics, future treatments, and systems using real-time health data.
A team of Western University researchers has discovered a potential path toward a cure for amyotrophic lateral sclerosis (ALS) by targeting an interaction between two proteins. The breakthrough, fueled by the Temerty Foundation's $10-million investment, could lead to treatment in human clinical trials within five years.
Researchers found a significant association between storing chemicals in attached garages and increased ALS risk. The study suggests that volatile chemicals released into the living space may contribute to this link.
A traumatic brain injury quadruples the risk of developing dementia and neurodegenerative diseases like ALS. USC scientists used lab-grown human brain structures called organoids to study TBI's effects. They identified a gene, KCNJ2, that helps protect nerve cells against injury.
Columbia University has received a $15 million grant to develop tailor-made gene-based therapies for nine people with rare forms of amyotrophic lateral sclerosis (ALS). The Silence ALS program will use antisense oligonucleotide (ASO) technology to silence genes that produce toxic proteins in motor neurons. The project aims to create pe...
Researchers identify 'junk proteins' as possible cause of ALS, a degenerative disease characterized by motor neuron death. The accumulation of these proteins is linked to the aging process, suggesting a new hypothesis for understanding ALS.
A recent study published in Cell reveals significant similarities between amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), two distinct neurological disorders. The research found shared gene expression patterns across various brain regions, suggesting potential therapeutic targets for both conditions.
A study by Michigan Medicine found that recreational activities such as golfing, gardening, and woodworking may increase the risk of developing amyotrophic lateral sclerosis (ALS) in men. The study surveyed over 700 individuals with and without ALS to assess their hobbies and non-work related activities.
Scientists developed an innovative neural cell culture model that replicates the aberrant behavior of TDP-43 in neurons. The model identified NPTX2 as a toxic protein that accumulates in cells containing abnormal TDP-43, leading to neurodegeneration in ALS and FTD.
Scientists have mapped out the proteins involved in motor neurone disease (MND) across its trajectory, identifying potential therapeutic pathways for further investigation. The study found that a protein-folding factor called DNAJB5 is elevated early on in MND, sparking curiosity about its role in disease progression.
A novel drug principle has been successfully tested in a mouse model and brain organoids of ALS patients, preventing cell death and improving motor abilities. The discovery of the TwinF interface inhibitor FP802 offers a promising path for fighting ALS and could lead to the development of effective treatments.
ATH-1105, a small molecule positive modulator of the neurotrophic HGF system, demonstrates significant neuroprotective effects and extends survival in preclinical models of ALS. The study highlights the therapeutic potential of ATH-1105 in slowing or stopping neurodegeneration.
A phase II clinical trial found Muse cell-based product CL2020 to be highly tolerated and improved ALS symptoms, but may not halt disease progression. The treatment's efficacy depends on combining it with other drugs for future treatments.
A novel cyclic thiosulfinate cross-linker has favorable drug-like properties and can stabilize the SOD1 dimer in vivo, indicating potential therapeutic benefits for fALS. This study uses a mouse model to evaluate protein cross-linking as a strategy to treat SOD1 variants associated with familial ALS.
A new study in the Canadian Medical Association Journal found that organ donations after medical assistance in dying (MAiD) represented 14% of all deceased organ donations in Quebec in 2022. The majority of donors had neurodegenerative diseases, with amyotrophic lateral sclerosis being the most common condition.
Researchers at Nagoya University have discovered a relationship between ALS progression and the disruption of mitochondria-associated membranes (MAM) and TBK1 activity. Decreased activation of TBK1 is linked to motor neuron death in ALS patients and mice with disrupted MAM.
Research suggests that structural changes in upper motor neurons send a signal to immune cells, leading to toxic effects on neurons and reduced synaptic connections. Blocking inflammation with a semi-synthetic drug can restore synaptic connections and improve ALS symptoms.
A team of scientists identified VAP as a molecular anchor that stabilizes mitochondria near synapses in dendrites, supporting memory formation and plasticity. The discovery links VAP to ALS-linked protein and suggests that mitochondrial stabilization is critical for neuronal function and health.
A study found increased brain inflammation and structural changes in special operations personnel exposed to blasts, potentially increasing the risk of long-term brain-related disease. The research suggests that repetitive exposure to low-level blasts may cause brain injuries and highlights the need for better protective measures.
Researchers found that misfolded prion proteins can trigger the clumping of TDP-43 in nerve cells, leading to reduced splicing activity and altered protein expression. This study reveals a new mechanism of how disease-associated prion proteins affect physiological signaling pathways through cross-seeding.
A molecular trap prevents toxic Poly-GR dipeptide compounds from causing harm to neurons, reducing death in ALS patients and animal models. The study demonstrates promising results for protecting neurons from abnormal proteins generated in other neurodegenerative diseases.
Researchers have identified BMAA, a chronic neurotoxin linked to ALS and Alzheimer's, in dust particles from the Great Salt Lake. This dust poses an environmental health risk due to its inhalation potential.
Dr. Vidhya Rangaraju has received a $1.2 million grant from the Chan Zuckerberg Initiative to investigate disrupted energy supply in neurons causing cognitive decline in ALS. Her lab will use super-resolution microscopy and biosensors to study metabolic disruptions in ALS.
Researchers developed a drug that triggers the destruction of damaged mitochondria, restored motor function in aging mice with ALS-like symptoms. The results show promising promise for treating neurodegenerative diseases like Parkinson's and Alzheimer's disease.
Researchers developed an environmental risk score to assess ALS risk and survival using a blood sample, associating several individual pollutants with disease risk. A mixture of pesticides in the blood significantly increased ALS risk, with those in the highest group having twice the risk as those in the lowest.
A new national ALS research consortium, Access for All in ALS, has been launched with a $16.7 million NIH award. The consortium will include 34 clinical sites nationwide and generate a longitudinal biorepository linked to detailed clinical information.
Dr. Qihui Zhou aims to investigate the role of immune cells in ALS and FTD with a focus on the C9orf72 gene mutation, potentially leading to better treatments.
A promising drug candidate CDNF has been found to prolong the lifespan of ALS patients and alleviate symptoms in rats and mice. The study suggests that CDNF may rescue motoneurons by reducing ER stress response and cell death.
A recent study found that over 70% of ALS patients and their caregivers are not disaster-prepared, highlighting the need for improved measures. The research also revealed significant gaps in equipment and knowledge among respondents, including ventilator users.
The new guidelines provide a framework for genetic testing and counseling, recommending comprehensive testing and gene-targeted therapies for all persons with ALS. The guidelines aim to improve access and standardize practice among neurologists and genetic counselors.
Researchers at the University of Sydney have developed a nanoscale optical technique to monitor protein aggregates forming in cells, which can lead to neurodegenerative diseases such as Alzheimer's and ALS. The study provides a new window into the transition of proteins from liquid to solid phase.
Scientists have successfully restored damaged nerve-to-muscle connections in a highly aggressive mouse model of amyotrophic lateral sclerosis (ALS) using grafted replacement motor neurons and optical nerve stimulation. The treatment improved muscle contraction force by over 13-fold, suggesting its potential for treating ALS patients.
Researchers at OHSU identified a type of monoclonal antibody that can slow the progression of amyotrophic lateral sclerosis (ALS) by modulating immune cells. The study used a mouse model and confirmed results in human brains affected by ALS, revealing a promising potential treatment for this fatal neurodegenerative disease.
A study published in Neuron reveals that hundreds of proteins and mRNA molecules are found in the wrong place in nerve cells affected by Motor Neurone Disease, a condition that causes paralysis. The researchers found that mislocalisation affects many more proteins than first thought, especially those involved in RNA binding.
New research suggests targeting autoimmune inflammation associated with ALS could be a promising approach. The study found that two drugs, including one already approved for multiple sclerosis, reduced the expression of inflammatory proteins in ALS patients' immune cells.
Researchers found that people with ALS who consume more omega-3 fatty acids like alpha-linolenic acid have a slower physical decline, a slightly extended survival, and lower risk of death. Omega-6 fatty acids like linoleic acid also showed promise in reducing mortality risk.
A new study led by Harvard T.H. Chan School of Public Health found that consuming omega-3 fatty acids, particularly alpha-linolenic acid, may help slow the progression of disease in patients with amyotrophic lateral sclerosis (ALS). Higher blood levels of ALA were associated with a slower disease progression and lower risk of death.
Researchers developed a polygenic scoring system to predict ALS disease risk, improving case status prediction in Michigan and Spain. The system takes into account common genetic variants and explains 4.1% of ALS cases caused by genetic factors.
Research at Umeå University reveals unique muscle fibers in extraocular muscles are resistant to ALS and increase in proportion when other fibers decrease. This finding could lead to new treatment strategies for slowing down the disease.
Researchers developed MOVA to evaluate the pathogenicity of missense variants based on their structural position. The new method showed superior performance in predicting pathogenicity, especially for hotspot mutations in ALS-causing genes like TARDBP and FUS.
A study by CU Boulder researchers suggests that the ancient virus-like protein PEG10 may contribute to the development of ALS by interfering with brain and nerve cell communication. The findings provide a promising new target for treating the underlying cause of the fatal disease.
Researchers found that ropinirole is safe and effective in slowing ALS progression, with patients showing improved physical activity and muscle strength after treatment. The study suggests that the method of growing motor neurons from patient-derived stem cells could be used to predict a patient's response to the drug.
Researchers found reduced editing efficiency of GluA2 mRNA in ALS patients' cerebrospinal fluid, correlating with longer disease duration and advanced symptoms. This discovery could lead to the development of therapies targeting RNA editing for treatable ALS cases.
A study published in Neurobiology of Disease suggests that targeting the sigma-1 receptor and ATAD3A protein may prevent mitochondrial dysfunction in ALS. The researchers found that this approach could lead to a novel therapeutic strategy for neurodegenerative diseases.
Researchers genetically engineered neural progenitor cells to release a neuroprotective protein, preventing neuron death in animal models of ALS and retinal disease. Engineered NPCs successfully preserved vision in rats with retinal disease.
Cedars-Sinai investigators have discovered a novel way to treat amyotrophic lateral sclerosis (ALS) and retinitis pigmentosa using human induced pluripotent stem cells. The new approach uses cells derived from iPSCs that are renewable, scalable, and can delay disease progression in rodents.
Researchers are investigating whether faecal microbiota transplantation can modulate the immune reaction and alter gut microbiota in early-stage ALS patients. The trial aims to investigate the relationship between specific gut bacteria and their action on immune system cells.
A simple blood test for ALS has been developed using microRNA analysis, which can accurately identify patients with the disease. The test uses a unique protein called L1CAM to concentrate diagnostic particles from extracellular vesicles in the bloodstream.
Research reveals that cold activates cellular cleansing mechanisms that break down protein clumps, preventing age-related diseases like Alzheimer's and Parkinson's. By modulating proteasome activity, scientists have found a potential therapeutic target for aging and related neurodegenerative disorders.
Researchers have created wearable microscopes to produce high-definition, real-time images of mouse spinal cord activity across previously inaccessible regions. This technology enables unprecedented insight into the neural basis of sensations and movement in healthy and disease contexts.
A team of researchers has discovered a protein deficiency in the neurons of patients with neurodegenerative diseases, such as ALS and Alzheimer's disease. They have developed an approach to fix this deficiency using antisense oligonucleotides that can correct abnormal RNA processing and restore stathmin-2 protein levels.
The American College of Chest Physicians released a clinical practice guideline on respiratory management for patients with neuromuscular weakness, providing evidence-based recommendations for mouthpiece ventilation and airway clearance therapies. The guideline aims to improve care for this vulnerable population.
The study reveals that gasdermin E drives changes in neurons that contribute to disease progression, leading to mitochondrial damage and axon degeneration. Inactivating gasdermin E prevents cellular damage and delays the progression of ALS in mice.
A study found that RNA methylation plays a pivotal role in TDP-43-related neurodegeneration in ALS. The researchers observed highly abundant RNA methylation in the end-stage tissues of patients with ALS. This discovery opens up new avenues for research into the disease, which is linked to environmental exposure.
Researchers have generated large-scale muscle-controlling nerve cells from ALS patients, revealing striking differences in gene expression between males and females. The study, published in Neuron, used over 450 lines of stem cells to create motor neurons that can potentially lead to the development of new therapeutics.
Researchers at USC Keck School of Medicine have identified two new avenues for treating diverse forms of ALS by suppressing genes and inhibiting proteins. The findings suggest that targeting SYF2 gene suppression and PIKFYVE kinase inhibition may lead to broadly effective treatments for the disease.
A team of researchers from Harvard and MGH developed a soft robotic wearable capable of significantly assisting upper arm and shoulder movement in people with ALS. The device improved range of motion, reduced muscle fatigue, and increased performance of tasks like holding or reaching for objects.
Scientists have developed a technique to detect RNA structures in live cells, shedding light on the role of G-quadruplexes in neurodegenerative diseases. The method uses fluorescent spectroscopy and resolves existing limitations in studying these structures in real-time.
A new study has identified distinct patterns of circular RNA expression in human ALS muscle tissue, which display disease-specific gradients and could inform about neuromuscular molecular programs in ALS. The research reveals that specific circRNAs are elevated in ALS muscle biopsies but reduced in spinal cord samples from ALS patients.
Scientists at the CRCHUM have identified a protective probiotic for ALS, Lacticaseibacillus rhamnosus HA-114, that prevents neurodegeneration in the C. elegans worm model. The probiotic helps reduce motor disorders and restore balance to impaired energy metabolism, leading to a decrease in neurodegeneration.