Articles tagged with Amyotrophic Lateral Sclerosis
Timothy M. Miller receives award for work on effective therapies for ALS, developing method to turn off toxic protein production in brain and spinal fluid. His approach is currently being tested in human clinical trials.
Researchers at Syracuse University are making progress in understanding the disease mechanism of ALS by studying ubiquitin and Ubiquilin-2 protein interactions. They found that ubiquitin eliminates droplets of UBQLN2, a protein-encoding gene linked to ALS and dementia.
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.
Researchers studied angiogenin's entry into central nervous system cells to better understand its role in brain diseases like ALS and dementia. The study found that angiogenin uptake has multiple biochemical pathways, revealing a more intricate mechanism than initially thought.
A study suggests that frequent exposure to diesel exhaust may increase the risk of amyotrophic lateral sclerosis (ALS), with a stronger link observed in men exposed for more than 50% of their working life. The research, published in the American Academy of Neurology's meeting program, found no associations among women, highlighting the...
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 Western University have discovered a unique neurobiological pathway triggered by head trauma that underlies both Chronic Traumatic Encephalopathy (CTE) and Lou Gehrig's disease. The study, published in the January 2018 issue of Neurology, identifies a common link between CTE and ALS-related cognitive impairment.
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.
A study published in Nature Communications reveals a genetic mutation in the C9orf72 gene causes a vicious cycle of toxic protein production, driving neuronal death in ALS. The researchers found that cell stress activates more toxic protein production, creating a loop that potentially drives disease progression.
Researchers have identified multiple misfolded pathways and intermediate states in the protein superoxide dismutase-1 (SOD1), a key player in ALS. Understanding these pathways is crucial for developing targeted therapeutic interventions.
Researchers studied 3000 human neuromuscular junctions, revealing details of their anatomy and structure. Human NMJs were found to be smaller and frailer than those in mice and rats, with no effect of age on their health.
Researchers discovered a new mechanism to prevent RNA from leaving cells' nuclei, leading to fresh treatment approaches for the most common form of motor neurone disease. By targeting SRSF1 protein, it's possible to reduce rogue RNA molecules and open up new areas of investigation for gene therapy.
A study published in Neurology found that approximately 17% of ALS cases are caused by gene mutations, with rare and likely harmful variants being more common in those with the disease. The research highlights the importance of genetic factors in ALS development and suggests a larger percentage of sporadic cases may be linked to genetics.
A new study has found that a decades-old malaria drug can safely lower levels of a toxic protein linked to familial ALS. The research suggests the drug could potentially slow disease progression in patients with genetic mutations.
A molecule called polyGP accumulates in the CSF and blood cells of patients with C9ORF72 ALS, offering a potential pharmacodynamic marker for therapeutic responses. Detection of polyGP in asymptomatic carriers may help identify patients at risk before symptoms appear.
Research published in Occupational & Environmental Medicine found a doubling of ALS risk among men exposed to very low frequency electromagnetic fields at work. High levels of exposure were associated with nearly twice the likelihood of developing the disease.
A new study suggests that consuming fish and seafood with higher levels of mercury may increase the risk of amyotrophic lateral sclerosis (ALS). The study found that individuals who ate fish and seafood regularly were at double the risk for ALS compared to those with lower mercury intake.
Researchers found that children of parents with C9orf72 mutations are more likely to develop frontotemporal dementia and ALS at a younger age, without accelerated disease progression. The study suggests that the length of the C9orf72 repeat is determinative for the age of onset of the disease.
A team of biomedical scientists has identified a molecule that targets the EphA4 receptor, a gene associated with rapid ALS progression. The research aims to delay motor neuron death and explore potential applications in spinal cord injury, Alzheimer's disease, and some cancers.
Researchers at the University of Illinois College of Medicine found that butyrate corrected a gut microbiome imbalance and reduced gut leakiness in mice with ALS. The treated mice lived longer than control mice and showed improved neuromuscular function.
A naturally occurring growth factor called FGFBP1 is secreted by muscle fibers to maintain neuromuscular junctions. However, ALS patients have reduced levels of FGFBP1 due to the accumulation of TGF-beta, a growth factor associated with the immune system.
Researchers have developed a groundbreaking 3D imaging technique that maps the reorganization of genetic material in cell nuclei, providing a new understanding of how chromosomes are compacted and genes are activated or silenced. This breakthrough uses X-ray microscopy to visualize the structure and movement of chromatin in real-time.
The study found that the GSK3 enzyme regulates the persistent sodium current, which affects a nerve cell's excitability and firing activity. This discovery may lead to chronotherapeutics, where treatments are tailored to the time of day to maximize health benefits and minimize side effects.
High physical fitness increases ALS risk before age 45, while individuals with high IQs have increased risk at 56+ years. Individuals with lower BMI (25+) show reduced risk across all ages.
Researchers have developed two new mouse models of amyotrophic lateral sclerosis (ALS) that exhibit protein clumping and display clinical features seen in patients. The models may help scientists better understand the disease and develop new treatments.
A new study found that antioxidant nutrients, fruits, and vegetable intake are associated with better ALS function at baseline. The analysis of 302 ALS patients suggested promoting fruit and vegetable intake to support nutritional care.
A major new study aims to uncover the secrets of ALS through research in Latin America, examining if mixed ancestry has a protective effect on developing the disease. The project will establish registers of incidence, prevalence, and risk factors for ALS in Cuba, Chile, and Uruguay.
Researchers discovered that RIPK1 damages neuronal axons by disrupting myelin production, leading to axonal degeneration and hallmark ALS symptoms. The findings suggest that inhibiting RIPK1 activity may halt the progression of ALS and provide new therapeutic options.
A groundbreaking study found that keeping a specific RNA binding protein, TDP-43, from moving inside nerve cells can prevent cell death and disease progression in ALS and other neurodegenerative disorders. The researchers developed small proteins to block the protein's localization, preventing toxicity and disease progression in mice.
Researchers have identified three new risk genes for amyotrophic lateral sclerosis (ALS) using genetic data from Project MinE. One of these genes, C21orf2, increases the risk of developing ALS by 65%. The study's findings could aid the development of personalized treatments using gene therapy.
Scientists from the University of Sheffield have discovered a novel function of the C9orf72 protein, which is linked to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The study found that the C9orf72 protein regulates the initiation of autophagy, a vital process for nerve cell survival.
Researchers found that transplanting human stem cells into the spinal cord was done safely, but did not slow down the progression of the disease. The treatment resulted in temporary side effects, including pain and swelling, but no conclusive evidence of effectiveness was found.
Researchers have found that measuring copper and zinc concentrations in blood and tissue may allow for early diagnosis of Amyotrophic Lateral Sclerosis (ALS). The study, presented at the Goldschmidt conference, showed significant changes in these elements prior to symptom onset.
Researchers at Umeå University have discovered that aggregated SOD1 protein in motor neurons causes rapid spread of ALS in mice. The study suggests a domino effect that spreads the disease up the spinal cord, mirroring human cases with hereditary traits for ALS.
Researchers created a mouse model that closely replicates the symptoms and gene expression patterns of ALS and frontotemporal dementia. The model allows scientists to understand how the C9orf72 gene mutation causes paralysis and dementia, and potentially develop treatments.
Lauren Sciences will use the grant to customize LAUR-301 with neurotrophic factor and deliver it to disease sites in central nervous system ALS mice. The company aims for LAUR-301 to enter human clinical trials and become a transformative V-Smart TM Nanomedicine for treating ALS.
Researchers at Oregon State University have developed a therapy that halts the progression of ALS in mice for nearly two years, allowing them to approach their normal lifespan. The treatment uses copper-ATSM, which delivers copper specifically to cells with damaged mitochondria and has low toxicity.
A team of researchers discovered a protein called Pur-alpha that can protect against toxic degeneration in cells, which may lead to a treatment for ALS. The study suggests that Pur-alpha could serve as a novel therapeutic target for developing a treatment for ALS patients.
Matthew D. Disney's innovative approach uses cells as reaction vessels to synthesize treatments within disease-affected cells, offering highly specific and precise therapies. This technology has potential applications in treating over 30 incurable diseases, including ALS, fragile X syndrome, and Huntington's disease.
Researchers at Barrow Neurological Institute and TGen will analyze blood and CSF samples from 60 participants to identify peptide, protein, and RNA biomarkers indicative of ALS progression. The goal is to develop effective treatments for patients in clinical trials.
Researchers found that the most common genetic defect in ALS causes nuclear pore dysfunction, leading to cell death. This discovery empowers the search for genetic causes of sporadic ALS and offers new hope for treatment options.
Researchers developed a fly model to study age-dependent neurodegeneration at single cell resolution, identifying three genes involved in the process. The findings have relevance for understanding ALS progression and could lead to therapies for neurodegenerative diseases.
Researchers found that groups of neurons work together to signal muscles about when and where to move, firing in complex rhythms. The study aims to create prosthetic devices that better understand and respond to a person's thoughts.
Scientists at the University of Montreal have discovered a new approach to treating amyotrophic lateral sclerosis (ALS) by targeting the immune system. The study, published in Nature Communications, found that an imbalance of the immune system can contribute to ALS and trigger motor neuron destruction.
Researchers at the University of Toronto have identified a possible way to predict if an individual is genetically prone to ALS by analyzing DNA repeats in the C9orf72 gene. The study found that a certain number of repeats, when modified with methyl groups, can cause the disease.
Researchers at Mayo Clinic developed a mouse model exhibiting neuropathological and behavioral features associated with C9ORF72 mutation. The study suggests a link between the repeat expansion in C9ORF72 and TDP-43 pathology, potentially leading to therapeutic agents targeting RNA foci and c9RAN proteins.
Hristelina S. Llieva will receive a two-year Clinical Research Training Fellowship in ALS to investigate glial cell dysfunction contributing to familial ALS. The award supports her work on the C9ORF72 linked subtype of familial ALS, which is viewed as a predominantly neuronal disease.
Scientists have found that senataxin, a protein associated with neurodegenerative diseases, plays a key role in the body's natural antiviral response. Abnormal inflammation generated by senataxin deficiency may contribute to disease progression in conditions like ALS.
A recent study has identified a group of gene variants that may contribute to sporadic motor neurone disease, a devastating condition affecting thousands of Australians. Researchers sequenced the protein-coding genes of 44 MND-affected individuals and their parents, finding rare genetic changes in two-fifths of cases.
Researchers have identified a new gene, TBK1, associated with sporadic ALS, highlighting the importance of inflammation and autophagy pathways. The study also found that OPTN may be a major player in the disease, interacting physically and functionally with TBK1.
Researchers found 26% of sporadic ALS patients carry potential mutations in known ALS genes, suggesting genetic errors contribute to significantly more cases. Mutations in multiple genes can accelerate disease onset.
Researchers found that blocking the ALS mutation in the brain slowed disease onset and progression, suggesting that early dysfunction of brain motor neurons may contribute to later disease development. The study used rats with ALS, showing delayed disease onset and extended survival when suppressing the mutation in the brain.
Researchers found that aging astrocytes lose ability to protect motor neurons, but replacing old cells with engineered protein improves neuron survival. A specially engineered protein called GDNF increases motor neuron survival when cultured with aging astrocytes.
Researchers identified the TUBA4A gene as associated with familial amyotrophic lateral sclerosis (ALS), a fatal neurological disorder. The mutated protein weakens the microtubule network, leading to motor neuron death and paralysis.
A new study suggests that ALS progression is linked to increased protein instability, particularly in mutant superoxide dismutase (SOD) proteins. The researchers found that SOD clusters or aggregates are common in affected motor neurons and support cells, suggesting a potential target for therapy.
Rabies uses a nerve growth factor receptor to enter the central nervous system, where it causes acute inflammation and violent aggression. The virus manipulates neuronal transport machinery to move faster than normal, allowing it to reach the brain with maximum speed.
The team determined the high-resolution structure of Dbr1 enzyme, which breaks loops of ribonucleic acids and could be a new target for treating most cases of ALS. By inhibiting this enzyme's activity, researchers hope to prevent TDP-43 aggregates from forming in motor neurons.
The ALS Association granted Lauren Sciences LLC a grant to develop a V-Smart therapeutic for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. The project aims to deliver neurotrophic factors across the blood brain barrier using the company's novel V-Smart nanovesicle platform technology.
Researchers found that serum albumin and creatinine levels are related to ALS survival in both sexes. Lower albumin and creatinine levels are related to worse clinical function at diagnosis.
Researchers found a significant association between higher dietary intakes of total ω-3 PUFAs and reduced risk for ALS. Consuming both α-linolenic acid (ALA) from plant sources and marine ω-3 PUFAs contributed to this protective effect, while ω-6 PUFA intake was not linked to ALS risk.