Articles tagged with Neurodegenerative Diseases
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A Taiwanese study found that ACE-inhibitors use was associated with a 57% reduced risk of developing amyotrophic lateral sclerosis (ALS) in patients prescribed more than 449.5 cumulative defined daily doses. The research used data from over 14,500 individuals and suggested long-term exposure to ACEIs may be inversely linked to ALS risk.
Researchers developed two mouse models with mitochondrial distribution defects that mimic neurological problems similar to ALS. The study found that impaired movement and muscle clasp were symptoms of the disease, suggesting a link between mitochondria distribution and motor neuron disease progression.
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 have successfully treated a neurodegenerative disease in worms using resveratrol, which could lead to new treatments for humans. The disease, adult onset neuronal lipofuscinosis, affects only 1 in 100,000 people and has no known cure.
Neurons store glycogen as a rapid energy source, but excess leads to death. Conversely, small amounts are beneficial for cell survival during oxygen depletion.
Researchers at the Weizmann Institute of Science have identified a key player in triggering brain inflammation and nerve cell death in severe forms of Gaucher disease. This discovery may lead to new treatments, including those that can cross the blood-brain barrier to target neurological symptoms.
Activating P300 protein facilitates DNA repair in hippocampal neurons, potentially treating neurodegenerative disorders. Researchers propose novel object recognition tasks as a therapeutic complement to classical drug-based therapy.
A 2-year study found diflunisal reduced neurological impairment and preserved quality of life in familial amyloid polyneuropathy patients compared to placebo. The treatment showed potential benefit in a phase 1 study and is well-tolerated by patients.
Researchers discovered that Neurexin2 dysfunction contributes to neurodegeneration in Spinal Muscular Atrophy, a devastating genetic disease affecting infant and young adults. A restoration of Neurexin2 activity partially recovers neuron function in SMN deficient zebrafish, offering a new direction for therapy of neurodegeneration.
5-hydroxymethylfurfural, extracted from wine-processed Fructus corni, protects against H2O2-induced rat hippocampal neuron apoptosis by regulating apoptosis-related genes. This finding provides evidence for the clinical prevention and treatment of oxidative injury-related diseases in the brain.
Researchers have identified a link between VCP gene mutations and toxic protein buildup in ALS patients, providing a new approach for developing treatments against the devastating disease. The study suggests that activating autophagy to clear stress granules could offer a new strategy for treating neurodegenerative diseases.
A team of researchers has identified a gene mutation responsible for a severe form of pontocerebellar hypoplasia, a currently incurable neurodegenerative disease affecting children. A nutritional supplement, AICAR, bypasses the block that prevents protein synthesis, restoring neuronal survival.
Researchers discover histone H1 protein's role in neurodegenerative disease and stroke; protein can trigger immune response and drive brain cell death. The study may lead to the development of new treatments for neurodegenerative diseases and stroke.
A new gene therapy treatment has been developed to cure Sanfilippo Syndrome type A in animal models by introducing the sulfamidase enzyme into cells, eliminating glycosaminoglican accumulation and associated neuroinflammation. The treatment has shown promising results in mice and dogs, with improved behavior and life expectancy.
Researchers discovered a previously unrecognized organelle gatekeeper function in C. elegans, which restricts the flow of Golgi and endosomal organelles into axons. Additionally, two studies identified novel players in meiotic silencing by unpaired DNA in Neurospora, providing insights into genome integrity and transmission.
Researchers at the University of Illinois Chicago have found that a unique modification to microtubules in neurons makes their cytoskeleton singularly robust. This discovery may help guide the search for treatments for neurodegenerative diseases.
Researchers at CSHL found that transposons increase in abundance and activity in the brains of fruit flies as they age, correlating with neurological degeneration. This 'transposon storm' may contribute to age-related brain defects in humans.
Researchers identify C9orf72 mutation as cause of ALS and FTD, finding it creates an RNA sponge that soaks up Pur alpha protein, leading to neurodegeneration. The study provides insight into the disease mechanism and suggests a potential therapeutic strategy targeting the toxic RNA or its interaction with Pur alpha.
Research reveals that VCP gene mutations disrupt energy production in cells, leading to mitochondrial dysfunction and increased vulnerability. This study sheds new light on the role of VCP in multiple neurological disorders.
Researchers identified an enzyme that removes ADP-ribose modifications from proteins, essential for normal cell function. This finding provides a new pathogenic mechanism for neurodegenerative disorders and may lead to the development of new cancer drugs.
A new virtual reconstruction of human metabolism, Recon 2, has been built to identify causes of diseases and develop new treatments. The model provides a detailed map of metabolic pathways, allowing researchers to pinpoint specific gene expressions and their effects on disease progression.
Researchers have found new candidate disease proteins for neurodegenerative disorders, including two RNA-binding proteins with prion-like segments associated with inherited forms of ALS and multisystem proteinopathy. Mutations in these proteins accelerate the formation of self-organizing fibrils that contribute to disease.
Scientists will use high-throughput screening to identify novel inhibitors of ASK1, a promising therapeutic target for diseases like rheumatoid arthritis. The grant aims to develop new treatments using compounds from the Scripps compound collection.
Researchers discovered a new mechanism by which STAT3 helps prevent axon degeneration, a hallmark of neurodegenerative diseases. CNTF treatment stimulated STAT3 to inhibit stathmin, leading to increased axon growth and reduced breakdown in ALS patients.
The Wellcome Trust has announced its first Pathfinder Awards to support research into rare and orphan diseases, including Neurodegeneration with Brain Iron Accumulation (NBIA) and Homocystinuria. The awards aim to facilitate collaboration between academia and industry to develop innovative treatments for these debilitating conditions.
Dr. Patricia Dickson's research aims to develop a stem cell-based therapy for mucopolysaccharidosis I, a fatal pediatric disease causing neurodegeneration. The project seeks to address critical issues in the development of therapeutic candidates.
Research by IRB Barcelona scientists reveals that glycogen accumulation in neurons is toxic, causing brain damage and reducing lifespan in both flies and mice. This finding has significant implications for understanding Lafora progressive myoclonic epilepsy and other neurodegenerative diseases.
A study discovered mutations in the mitochondrial methionyl-tRNA synthetase gene that cause neurodegenerative disorders in both fruit flies and humans. The findings suggest that antioxidants may counteract the negative consequences of these mutations in flies, raising hope for potential therapeutic approaches in human patients.
A study found that blocking a protein called AMPK helps increase animal mobility and reduce nerve cell death in animal models of amyotrophic lateral sclerosis (ALS). The findings suggest energy deficits may contribute to neurodegenerative disorders like ALS, offering new directions for treatment.
A team of researchers has associated mutations in the reticulon 2 gene with hereditary spastic paraplegia type 12. They discovered that these mutations likely cause neurodegeneration by disrupting ER-shaping proteins, providing new insights into this complex disorder.
A study published in Neuron reveals that upper and lower white matter fiber pathways play distinct roles in language processing. Patients with damage to the lower pathway struggle with lexical semantics but excel at constructing sentences, while those with damage to the upper pathway have difficulties with syntactic processing.
Researchers have discovered that a mutation in the CLN3 gene disrupts protein trafficking, leading to lysosome overflow and neuronal death in Batten disease. The findings may form the basis for a new therapy by targeting the kinase function of CLN3.
A new human genetic mutation, C9ORF72 repeat expansion, is identified as the most common cause of ALS and frontotemporal dementia. This mutation explains at least a third of all familial cases in Europeans and nearly half of all cases in Finland.
Researchers have pinpointed a new genetic segment on chromosome 9 as the cause of familial motor neuron disease, with affected patients carrying hundreds of DNA repeats that disrupt multiple mechanisms in motor nerve cells. This discovery may lead to new blood tests for early detection and treatment avenues.
Researchers at the University of Illinois have developed a new set of building blocks for simple synthesis of complex molecules. They successfully synthesized synechoxanthin, an antioxidant molecule, using their iterative cross-coupling method, which enables the creation of a whole new class of building blocks.
Researchers at Institut de recherches cliniques de Montréal have identified a key molecule directing axons during neural circuit formation. VEGF plays a crucial role in attracting nervous system axons, offering potential for therapies to re-grow axons after spinal cord injuries.
The study identifies thousands of sites of DNA methylation in a large set of physiological and pathological tissues, providing insight into human body processes and disease. The research may lead to finding the unknown origin of metastasis and developing more effective therapies.
A study published in Archives of Otolaryngology – Head & Neck Surgery found that patients with a reduced ability to smell or detect odors seem to adapt to their condition. They report fewer olfactory-triggered emotions and memories, using their sense of smell less in daily life.
A team at UCSF has identified the right hemisphere's pregenual anterior cingulate cortex as crucial for embarrassment. The study found that people with neurological damage in this region responded less emotionally to criticism, suggesting a potential diagnostic tool for neurodegenerative diseases.
A new study has uncovered the role of Sortilin in regulating Progranulin levels in neurons, which may hold promise for treating frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The research found that Sortilin-mediated PGRN endocytosis is a key pathway underlying FTLD-TDP pathophysiology.
The OHSU Doernbecher team is conducting a second phase of a landmark clinical trial to assess the safety and preliminary effectiveness of purified human neural stem cells as a potential treatment for infantile or late-infantile neuronal ceroid lipofuscinosis, a rare and currently fatal neurodegenerative disorder. The trial aims to eval...
The study identified mutations in the ataxin 2 gene as a genetic contributor to ALS, with expansions of glutamine in ataxin 2 linked to an increased risk for the disease. The findings may aid in the development of biomarkers and new therapies for ALS.
Researchers have created a genetically modified fruit fly that mimics key features of Charcot-Marie-Tooth disease, a neurodegenerative disorder. The study may reveal new information on how the disease develops in humans and provide a tool for discovering potential new drugs.
Resveratrol shows therapeutic potential in preventing age-related disorders, including neurodegenerative diseases, inflammation, diabetes, and cardiovascular disease. Low doses improve cell survival, while high doses increase cell death.
Researchers have identified a crucial molecular pathway required for the formation of brain neural circuits. This breakthrough has significant implications for understanding how axons reach their targets, paving the way for new therapies to treat spinal cord injuries, neurodevelopmental disorders, and neurodegenerative diseases.
A study by Berman et al. reveals that mitochondrial size and shape are regulated by fusion, fission, growth, and degradation processes. The researchers found that a protein called Bcl-xL manages the number, size, and energy-producing capacity of mitochondria.
A new study reveals that molecular motors in cells operate in a highly coordinated manner to move internal cargo and transport organelles. The findings provide insight into the mechanisms that instruct motor movement, potentially leading to therapies for neurodegenerative disorders such as ALS and Usher syndrome.
Researchers at UT Southwestern Medical Center identified a compound that liberates cholesterol trapped in cells, shedding light on its transport and suggesting a possible therapeutic target for NP-C disease. The findings show improved liver function and decreased neurodegeneration in mice treated with the compound.
Patients with pediatric-onset multiple sclerosis tend to have a higher yearly rate of relapses compared to those diagnosed in adulthood. This discrepancy may suggest greater plasticity and potentially more repair and remyelination in the younger nervous system.
Researchers have discovered a possible therapy for mucolipidosis type IV (ML4), a disease causing nerve cell death and progressive motor function loss in children. Introducing normal blood cells into genetically modified fruit flies with ML4 symptoms delayed cell death, suggesting bone marrow transplantation as a treatment option.
Researchers found that excessive glucose chains induce neuronal death and cause Lafora disease by disrupting glycogen levels. This discovery may lead to new treatment strategies for other neurodegenerative diseases.
Researchers found that defects in protein glycosylation, a process adding sugars to proteins, promote inflammation and neurodegeneration in MS. A dietary supplement similar to glucosamine may correct these defects and treat both short-term and long-term symptoms.
Researchers identify pur alpha protein as key player in FXTAS, a neurodegenerative disorder caused by CGG repeats in the FMR1 gene. The protein's sequestration leads to toxic brain aggregates and movement problems.
A four-year study investigates how dietary iron is used by cells, addressing endemic iron deficiency affecting 80% of the world's population. The researchers aim to understand cellular iron metabolism, a key process impacted in various human diseases.
Researchers identify mutations in moonlighting enzyme dihydrolipoamide dehydrogenase (DLD) that contribute to the reduction of frataxin production, leading to increased severity of Friedreich's ataxia. The study suggests DLD as a potential target for therapies of this condition.
U of MN researchers have shown that early brain development is tied to the severity of adult-onset neurodegenerative diseases like spinocerebellar ataxia type 1. The study suggests doctors may be able to target treatment to coincide with critical times in development.
Researchers found that imatinib can block the development of disease in a mouse model of rheumatoid arthritis, providing hope for a new treatment. Additionally, studying adrenomedullin's effect on female mice revealed that reduced expression may severely decrease fertility. Meanwhile, a study on TNF-alpha discovered that it decreases e...
Scientists at Duke University Medical Center have identified a gene defect linked to hereditary spastic paraplegia, a rare nervous system disease with no cure. The discovery could lead to the development of drugs targeting the defective gene and provide insights into other neurodegenerative diseases.
The American Institute of Ultrasound in Medicine has awarded a New Investigator Award to Dr. Elisa Konofagou for her groundbreaking work on treating neurodegenerative diseases using ultrasound-induced blood-brain barrier opening. Seven nominees presented their research, showcasing the diverse applications of ultrasound technology.
Researchers have developed a new classification system for myosins, increasing the number of subclasses from 18 to 24. This allows for better understanding of each myosin's function and its evolutionary links with other proteins.