ReviewNature reviews. Neurology2015
A new prion disease: relationship with central and peripheral amyloidoses.
Review in Nature reviews. Neurology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
31 citing papers in PubMed, 47 citations in OpenAlex.
- PrP turnover in vivo and the time to effect of prion disease therapeutics.PLoS pathogens · 2026Article
- GPI-anchorless prion disease is sensitive to oxidative stress and shows potential for treatment with edaravone, based on iPS-derived neuron study.Cellular and molecular life sciences : CMLS · 2025Article
- Revision and update of the position paper on the management of notifications of donors with Creutzfeldt-Jakob disease in Italy.Blood transfusion = Trasfusione del sangue · 2025Article
- Nonlinear characteristics of gait signals in neurodegenerative diseases.Frontiers in neurology · 2025Article
- Limbic system synaptic dysfunctions associated with prion disease onset.Acta neuropathologica communications · 2024Article
- Prion strains viewed through the lens of cryo-EM.Cell and tissue research · 2023Review
- Prion therapeutics: Lessons from the past.Prion · 2022Review
- Huntington's disease: lessons from prion disorders.Journal of neurology · 2021Article
- Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases.Scientific reports · 2021Article
- Genetic testing in dementia - utility and clinical strategies.Nature reviews. Neurology · 2021Review
- Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS.PLoS biology · 2020Article
- Surface-Directed Structural Transition of Amyloidogenic Aggregates and the Resulting Neurotoxicity.ACS omega · 2020Article
- Nano-scientific Application of Atomic Force Microscopy in Pathology: from Molecules to Tissues.International journal of medical sciences · 2020Review
- Cerebral Amyloid Angiopathy, Alzheimer's Disease and MicroRNA: miRNA as Diagnostic Biomarkers and Potential Therapeutic Targets.Neuromolecular medicine · 2019Review
- Structural features distinguishing infectious ex vivo mammalian prions from non-infectious fibrillar assemblies generated in vitro.Scientific reports · 2019Article
- Recent Advances in Understanding Mammalian Prion Structure: A Mini Review.Frontiers in molecular neuroscience · 2019Article
- Evaluating the causality of novel sequence variants in the prion protein gene by example.Neurobiology of aging · 2018Article
- Propagation and spread of pathogenic protein assemblies in neurodegenerative diseases.Nature neuroscience · 2018Review
- The emerging roles of protein homeostasis-governing pathways in Alzheimer's disease.Aging cell · 2018Review
- Two novelAnnals of clinical and translational neurology · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
Abstract
Prion diseases are typically recognized as rapidly progressive dementing illnesses that also feature myoclonus and cerebellar ataxia. Several families have now been described with a late-onset hereditary sensory and autonomic neuropathy caused by truncation of prion protein (PrP), and associated with systemic amyloidosis, which was a profoundly unexpected phenotype. The chronic symptoms of this disorder, termed PrP systemic amyloidosis, can be very disabling, and are comparable to familial amyloid polyneuropathy (FAP) caused by transthyretin mutations. Patients require symptomatic therapies directed towards control of nausea, diarrhoea, incontinence, neuropathic pain and postural hypotension. Although the potential transmissibility of this new prion disease is probably extremely low, we advocate PrP gene analysis before biopsy in the investigation of peripheral and autonomic neuropathies, or for patients with unexplained diarrhoea and neuropathy. Prion diseases and the FAPs both display prominent effects of mutation type on clinical presentation and patterns of pathology-a fascinating but unexplained observation. Several neurodegenerative diseases associated with central protein misfolding, such as Huntington and Parkinson diseases, also have under-recognized peripheral components. Most of the familial amyloidoses can be explained by known gene mutations, but amino acid variants in proteins involved in other central neurodegenerative diseases might direct the initial pathology to the periphery.
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What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.