Articles tagged with Prions
Researchers identified the prion protein as a key player in the progression of glioblastoma, a type of brain tumor. The study found that blocking the production of this protein using genetic editing reduced the ability of tumor stem cells to proliferate and invade tissues.
Researchers developed a gene-editing treatment that reduces prion protein levels in the brain by up to 60% and extends mouse lifespan by about 50%. The base-editing approach could lead to a one-time treatment for prion disease patients.
Researchers have developed new models to explore the role of a membrane anchor on the folding and aggregation of PrP. Anchoring stabilizes folding and inhibits aggregation, with clumping induced by pre-formed aggregates, suggesting a potential mechanism for infectious prion diseases.
Researchers found that Florida panthers are not at increased risk for contracting chronic wasting disease from eating infected deer. The study provides relief to conservation efforts, as the disease has spread through North America's deer and elk populations.
Researchers from Tokyo Medical and Dental University demonstrate a proof of concept for antisense nucleic acid therapy to prevent the spread of α-synuclein pathologies in synucleinopathies. The treatment, involving antisense oligonucleotides, effectively reduces Lewy pathology-like neuronal inclusion by over 90%.
A CHARMed collaboration has created a set of molecular tools called CHARMs that can turn off disease-causing genes, including those coding for the prion protein. The tools have shown promise in silencing the prion protein gene and improving or eliminating disease symptoms in animals.
A new NIH study using human cerebral organoids suggests a substantial species barrier preventing the transmission of chronic wasting disease from cervids to people. Researchers found no infection in healthy human cerebral organoids exposed to CWD prions for up to six months.
A new biomarker, miR-519a-3p, has been discovered to detect Alzheimer's disease in its asymptomatic stages. The molecule is linked to the expression of cellular prion protein, which is deregulated in people with neurodegenerative diseases.
Madhu Kannan, a University of Minnesota Medical School assistant professor, has been selected for the Chan Zuckerberg Initiative's Collaborative Pairs Pilot Project Awards to explore innovative approaches to understanding human prion diseases. The project will utilize cutting-edge approaches combining gene editing and neural activity i...
A new study from the University of Pennsylvania School of Veterinary Medicine sheds light on how Chronic Wasting Disease (CWD) impacts the gut microbiome, providing a potential tool for disease surveillance. Researchers found that certain types of gut bacteria can differentiate between CWD-positive and -negative deer.
Researchers have identified five cases of Alzheimer's disease acquired through exposure to contaminated cadaver-derived human growth hormone (c-hGH) used in medical treatment between 1959 and 1985. Biomarker analyses and autopsy results confirmed the diagnoses, highlighting a rare but significant risk of transmission via this route.
Researchers discovered that prion protein and copper form sticky droplets under oxidative stress, leading to abnormal solid formation. The study highlights the biological significance of liquid-liquid phase separation in regulating copper homeostasis by PrP.
Researchers at the University of Wisconsin-Madison found that ticks can harbor transmissible amounts of the protein particle causing CWD, potentially spreading the disease between deer. The study also suggests that better land stewardship and tick management could help manage CWD populations.
Scientists have used gene therapy to increase 'cold shock protein' levels in mice brains, protecting them against prion disease. The approach could lead to a safer treatment for acute brain injury and prevention of neurodegenerative diseases like Alzheimer's.
Researchers at the University of Pennsylvania's Working Dog Center trained dogs to distinguish between healthy and diseased deer feces. The dogs showed great specificity in detecting CWD-positive samples, suggesting a potential asset in managing the highly contagious disease.
Researchers have discovered that a specific mutation in the misfolding protein causing Parkinson's disease can also protect against multiple system atrophy (MSA), another fatal neurodegenerative disorder. The findings provide a promising lead for developing targeted treatments using personalized medicine approaches.
A recent study reveals that Down syndrome brains develop the same amyloid beta and tau prions as Alzheimer's disease, causing neurological dysfunction. With over 50% of people with Down syndrome developing Alzheimer's by age 40, this discovery offers new insights into the common underlying causes of these two diseases.
Studies on prion diseases in mice reveal coordinated gene expression changes before symptoms appear, shedding light on selective vulnerability and potential treatment targets. Researchers predict disease progression using new methods, suggesting therapies may be more effective when applied early.
Researchers have found evidence of genetic diversity in the prion protein gene of endangered Eld's deer, which could provide resistance to chronic wasting disease. The study suggests reducing the frequency of a variant associated with the disease and implementing strict management practices to prevent exposure.
A new study reveals the molecular mechanism that causes prion proteins to take on their pathological form, paving the way for possible therapeutic options. The discovery highlights the structure of the human prion protein and its intermediate forms, enabling the design of new organic molecules to block disease progression.
Researchers at Case Western Reserve University have identified the structure of protein fibrils linked to a hereditary form of human prion disease, revealing the mechanism for interspecies transmission. The study suggests that disease transmission between species can be predicted based on structural information.
Researchers discovered a new prion structure using electron microscopy, revealing key similarities and differences between distinct strains. This finding could lead to better understanding of how shape variations affect disease outcomes.
A five-year, $13 million grant will investigate the molecular and cellular mechanisms of prion transmission in CWD. The researchers aim to understand the inter-species transmission potential of the disease and its ability to spread from animals to humans.
Researchers have developed a monoclonal antibody called PRN100, which showed safe and encouraging results in treating Creutzfeldt-Jakob disease (CJD) in six patients. While the findings are preliminary and require further studies, they offer new prospects for this rare and fatal disease.
Multiple system atrophy (MSA) is a fatal neurodegenerative movement disorder with no effective treatments, progressing rapidly and impairing critical physiological functions. The researchers will investigate how misfolded protein aggregates contribute to disease pathogenesis using the NIH grant.
Researchers find that toxic aggregates form inside brain cells, causing axon damage and neuronal death. Silencing specific proteins can block the aggregation process, potentially preventing or treating prion diseases and other neurodegenerative disorders.
Researchers found two prion gene variants in Père David's deer that may reduce susceptibility to CWD. The genetic variants were surprising given the population's small founder size and conserved prion protein gene. Studies are needed to confirm whether these variants provide protection against CWD.
Researchers at Rice University have discovered a new mechanism by which prions can regulate protein synthesis in cells. The model proposes that prion aggregates and their monomers play a role in channeling RNA messages into new proteins, forming organized protein synthesis factories. This discovery has implications for our understandin...
A recent study published in eLife has discovered that prions, proteins known for their involvement in disease, may play a role in helping yeast cells cope with rapidly changing environmental conditions. The research found that prions can transform cell growth and survival, suggesting a new form of epigenetic control.
Case Western Reserve University researchers have identified structural elements on human prions that drive their replication in the brain. This breakthrough could lead to a new strategy to stop Alzheimer's disease and other neurodegenerative disorders, as scientists explore ways to bind to these sites to block progression.
A breakthrough discovery has pinpointed the mechanism by which normal proteins convert to diseased forms, causing conditions like CJD and Kuru. The research could lead to the development of new drugs to combat these devastating diseases.
A new study has sequenced the carbohydrate structures of glycoproteins from two different sheep prion strains, revealing no major differences that could explain the diversity in prion strains. The findings suggest glycans may not be responsible for the biochemical and neuropathological differences between strains.
A new study by NIH scientists reveals that prion disease affects the eye first, specifically targeting cone photoreceptor cells. The researchers used confocal microscopy to identify prion protein deposits in cones and rods, finding early damage in cilia and ribbon synapses.
Researchers at the University of California - Santa Cruz identify a specific segment of amyloid beta recognized by the cellular prion protein, which mediates its uptake into neurons and toxicity. This finding suggests targeting this process may be a promising approach for Alzheimer's drug development.
Researchers have discovered that pure prions do not exhibit neurotoxicity, but instead cause disease through a distinct species. Brain tissue from symptomatic prion-infected mice is found to be toxic to healthy neurons, highlighting the complexity of prion-induced neurodegeneration.
Scientists have found that a small proportion of individuals possess high levels of antibodies targeting the normal PrP version of the prion protein. These antibodies may be beneficial in targeting pathological aggregates for degradation by phagocytic cells, potentially offering new tools for studying neurodegeneration. The discovery s...
Researchers have discovered a promising new treatment strategy for patients with prion disease by reducing levels of prion protein. This approach has been shown to significantly extend the survival of lab animals and even reverse disease markers after toxic clumps have formed in the brain.
Researchers have developed a groundbreaking mouse model of human prion disease, demonstrating spontaneous formation of disease-relevant prion protein assemblies in mice with only human forms of the prion protein. This discovery is expected to provide valuable insights into human disease and inform the development of therapies.
Researchers at UMass Amherst have identified an unexpected role for prion nucleation seeds that enhances their ability to appear and resist curing. The minimum size of the seed complex determines whether the disease can persist, and controlling this transformation could lead to a cure.
Researchers used high-speed atomic force microscopy to visualize the formation of Sup35NM amyloid fibrils in real time. They discovered that oligomers and fibrils have distinct structural characteristics, challenging previous assumptions about their relationship.
Researchers found that prion proteins form liquid droplets modulated by DNA sequences, turning into solid and toxic states. These structures are related to Creutzfeldt-Jakob disease and other spongiform encephalopathies.
A team of scientists has developed a synthetic-molecule-based approach to isolate prion proteins in body fluids, paving the way for quick, noninvasive detection. The test also detects misfolded proteins associated with Alzheimer's and Parkinson's diseases.
A new study by NIH scientists found that a 40% household bleach solution can inactivate chronic wasting disease prions within 5 minutes, providing a potential method for decontaminating hunting and meat processing equipment.
Scientists have developed an experimental treatment using antisense oligonucleotides that slows the progression of scrapie, a degenerative central nervous disease caused by prions, in laboratory mice. Mice treated with the treatment lived 81% longer than untreated mice, with median survival times of up to 250 days.
A computational model has been developed to understand the mechanism of prion replication, which replicates in absence of genetic material. The study proposes a novel architecture consistent with recent experimental data and allows for the reliable prediction of protein conformational transitions.
Researchers have developed a human cerebral organoid system, or 'mini-brain,' to study sporadic Creutzfeldt-Jakob disease (CJD) and its subtypes. The model enables evaluation of potential therapeutics and provides insights into human prion disease progression.
Scientists have developed a new gene-targeted approach to study chronic wasting disease in mice, allowing for the detection of distinct prion strain properties in deer and elk. This breakthrough enables researchers to better understand the ability of prions to transmit disease between species.
Researchers detected and measured specific prion forms of amyloid beta (A-β) and tau proteins in human brain tissue, strongly associated with early-onset Alzheimer's disease. The findings suggest that active A-β and tau prions could drive the disease and prompt exploration of new therapies targeting prions.
Researchers discover gene TIA1 helps the brain keep fearful memories in check, reducing PTSD-like behavior in female mice. The study highlights key role of TIA1 in essential biological processes and offers promising new target for combating PTSD.
Researchers developed a new skin test to detect prion infections before symptoms appear, using RT-QuIC and sPMCA methods. The study demonstrates the preclinical utility of skin samples in detecting prions, which could serve as a biomarker for early diagnosis.
A UCL-led study confirms that some vials of discontinued hormone used in medical treatments contained seeds of a protein implicated in Alzheimer's disease. The research found that these seeds can seed amyloid pathology in mice and supports the team's hypothesis that amyloid beta was accidentally transmitted to patients via this treatment.
A major soil organic matter compound, humic acid, has been found to degrade chronic wasting disease prions and reduce their infectivity in mice. The findings suggest that soil organic material can break down the prions, making them less infectious.
Researchers used genetically modified mice with a highly permeable blood-brain barrier to test whether prions in blood can enter the brain. Their results suggest that passage of prions through the blood-brain barrier may not be relevant to disease development. Instead, prions likely reach the brain by traveling along nerves.
High levels of prions were found throughout the eyes of deceased sCJD patients, suggesting the eye as a potential source for early detection and prevention of disease transmission. Researchers also discovered that prion seeds accumulate in the retina, with implications for diagnostic tests before symptoms become apparent.
Scientists have discovered evidence of infectious prions in the eyes of deceased Creutzfeldt-Jakob disease (CJD) patients, which could lead to early diagnosis and treatment of this fatal neurodegenerative disease. The finding raises concerns about the safety of routine eye exams and corneal transplants.
A study found that prions, infectious proteins causing Creutzfeldt-Jakob disease, accumulate in the retina and other parts of the eye. The discovery could lead to new diagnostic tests and treatments for the disease.
New research reveals that not all prion strain combinations result in interference, suggesting greater strain diversity than previously thought. The study found that co-infecting strains amplify independently, changing the paradigm of prion strain interactions and implications for interspecies transmission.
Case Western Reserve researchers are investigating skin prions as a potential diagnostic biomarker for Creutzfeldt-Jakob disease. The study aims to determine if skin prions can serve as a pre-mortem and post-mortem diagnostic tool, as well as explore their role in transmission between humans.
Researchers developed a new assay to measure nucleation, the first step in prion transformation, allowing them to distinguish between proteins with prion behavior and those without. This approach may help understand prions associated with diseases as well as their role in normal biological processes.
New research found that replication under low RNA conditions alters disease features when transmitted back to hamsters, suggesting that RNA levels during replication influence prion adaptation. This study's findings may improve understanding of cross-species prion transmission and disease in mammals.