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References from Therapeutic strategies in prion disease: current evidence, translational challenges, and emerging directions. Local targets link to admitted publications; unresolved targets remain external evidence.
Incidence and predictors of adverse drug events in an African cohort of HIV-infected adults treated with efavirenz
10.11599/germs.2015.1075 · 2015 · External reference
Cross-linking cellular prion protein induces neuronal type 2-like hypersensitivity
10.3389/fimmu.2021.639008 · 2021 · External reference
Treatment with efavirenz extends survival in a Creutzfeldt-Jakob disease model by regulating brain cholesterol metabolism
10.1172/jci.insight.190296 · 2025 · External reference
Oral administration of repurposed drug targeting Cyp46A1 increases survival times of prion infected mice
10.1186/s40478-021-01162-1 · 2021 · External reference
CRISPR/Cas9-editing of PRNP in alpine goats
10.1186/s13567-024-01444-1 · 2025 · External reference
In vivo base editing extends lifespan of a humanized mouse model of prion disease
10.1038/s41591-024-03466-w · 2025 · External reference
The clinical drug candidate anle138b binds in a cavity of lipidic α-synuclein fibrils
10.1038/s41467-022-32797-w · 2022 · External reference
Rapidly progressive dementias and the treatment of human prion diseases
10.1517/14656566.2010.514903 · 2011 · External reference
Genetic aspects of human prion diseases
10.3389/fneur.2022.1003056 · 2022 · External reference
Symptomatic treatment, care, and support of CJD patients
10.1016/b978-0-444-63945-5.00021-0 · 2018 · External reference
Microglia-astrocyte communication via C1q contributes to orofacial neuropathic pain associated with infraorbital nerve injury
10.3390/ijms21186834 · 2020 · External reference
A naturally occurring variant of the human prion protein completely prevents prion disease
10.1038/nature14510 · 2015 · External reference
[18F]F-DED PET imaging of reactive astrogliosis in neurodegenerative diseases: preclinical proof of concept and first-in-human data
10.1186/s12974-023-02749-2 · 2023 · External reference
Evaluation of quinacrine treatment for prion diseases
10.1128/jvi.77.15.8462-8469.2003 · 2003 · External reference
Exploring the zoonotic potential of animal prion diseases: in vivo and in vitro approaches
10.4161/pri.28124 · 2014 · External reference
On the reactive states of astrocytes in prion diseases
10.1080/19336896.2021.1930852 · 2021 · External reference
PrPC knockdown by liposome-siRNA-peptide complexes (LSPCs) prolongs survival and normal behavior of prion-infected mice immunotolerant to treatment
10.1371/journal.pone.0219995 · 2019 · External reference
Microglia function during brain development: new insights from animal models
10.1016/j.brainres.2014.11.032 · 2015 · External reference
Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK
10.1111/j.1468-1331.2008.02108.x · 2008 · External reference
Microglia deficiency accelerates prion disease but does not enhance prion accumulation in the brain
10.1002/glia.24244 · 2022 · External reference
Diagnosis of Creutzfeldt-Jakob disease: effect of clinical criteria on incidence estimates
10.1212/wnl.54.5.1095 · 2000 · External reference
Microglia and prion disease
10.1002/jemt.1122 · 2001 · External reference
Strain-dependent morphology of reactive astrocytes in human- and animal-vole-adapted prions
10.3390/biom13050757 · 2023 · External reference
Mice devoid of PrP are resistant to scrapie
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Neuroinflammation, microglia, and cell-association during prion disease
10.3390/v11010065 · 2019 · External reference
Pathogenesis, detection, and control of scrapie in sheep
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Sulfated polyanion inhibition of scrapie-associated PrP accumulation in cultured cells
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A meta-analysis on RT-QuIC for the diagnosis of sporadic CJD
10.1007/s13760-021-01596-3 · 2021 · External reference
Unlocking Neuroinflammation: a balanced art for therapeutics of prion disease
10.1021/acschemneuro.4c00871 · 2025 · External reference
Activation of innate immunity and autophagy in brain tissues with prion disease and degradation of abnormal PrPs in cells - China's studies
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External reference
CNS-wide repopulation by hematopoietic-derived microglia-like cells corrects progranulin deficiency in mice
10.1038/s41467-024-49908-4 · 2024 · External reference
Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial
10.1016/s1474-4422(09)70049-3 · 2009 · External reference
Quinacrine does not prolong survival in a murine Creutzfeldt-Jakob disease model
10.1002/ana.10336 · 2002 · External reference
Clinical laboratory tests used to aid in diagnosis of human prion disease
10.1128/jcm.00769-19 · 2019 · External reference
Unresolved reference
2026 · External reference
Cellular pathogenesis in prion diseases
10.1051/vetres:2008021 · 2008 · External reference
Molecular mechanisms and biological functions of siRNA
10.59566/ijbs.2017.13048 · 2017 · External reference
The infection risk of intrathecal drug infusion pumps after multiple refill procedures
10.1111/j.1094-7159.2005.05218.x · 2005 · External reference
Specific inhibition of pathological prion protein accumulation by small interfering RNAs
10.1242/jcs.00494 · 2003 · External reference
The efficacy of tetracyclines in peripheral and intracerebral prion infection
10.1371/journal.pone.0001888 · 2008 · External reference
The role of microglia in prion diseases and possible therapeutic targets: a literature review
10.1080/19336896.2021.1991771 · 2021 · External reference
Diffusion-weighted MRI in sporadic Creutzfeldt-Jakob disease
10.1212/wnl.52.1.205 · 1999 · External reference
Scrapie-associated prion protein accumulates in astrocytes during scrapie infection
10.1073/pnas.88.2.375 · 1991 · External reference
Uptake and efflux of quinacrine, a candidate for the treatment of prion diseases, at the blood-brain barrier
10.1023/b:cemn.0000018617.21378.95 · 2004 · External reference
Treatment of transmissible spongiform encephalopathy by intraventricular drug infusion in animal models
10.1128/jvi.78.10.4999-5006.2004 · 2004 · External reference
Update on chronic wasting disease (CWD) III
10.2903/j.efsa.2019.5863 · 2019 · External reference
Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody
10.1073/pnas.151242598 · 2001 · External reference
Unresolved reference
2026 · External reference
Unresolved reference
External reference
Screening of 145 anti-PrP monoclonal antibodies for their capacity to inhibit PrPSc replication in infected cells
10.1074/jbc.m407006200 · 2005 · External reference
Tetracyclines affect prion infectivity
10.1073/pnas.162195499 · 2002 · External reference
Regional pattern of microgliosis in sporadic Creutzfeldt-Jakob disease in relation to phenotypic variants and disease progression
10.1111/nan.12461 · 2018 · External reference
Animal prion diseases: a review of intraspecies transmission. Open
10.5455/ovj.2021.v11.i4.23 · 2021 · External reference
Sporadic and familial CJD: classification and characterisation
10.1093/bmb/66.1.213 · 2003 · External reference
Leveraging the dominant-negative effect of the kuru-protective G127V prion protein variant as a novel therapeutic strategy
10.1016/j.nbd.2026.107506 · 2026 · External reference
Unresolved reference
2026 · External reference
A possible pharmacological explanation for quinacrine failure to treat prion diseases: pharmacokinetic investigations in a ovine model of scrapie
10.1038/sj.bjp.0706072 · 2005 · External reference
Divalent siRNA for prion disease
10.1093/nar/gkag287 · 2026 · External reference
Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease
10.1212/wnl.0b013e3182a9f3b4 · 2013 · External reference
Continuous quinacrine treatment results in the formation of drug-resistant prions
10.1371/journal.ppat.1000673 · 2009 · External reference
Polyclonal anti-PrP auto-antibodies induced with dimeric PrP interfere efficiently with PrPSc propagation in prion-infected cells
10.1074/jbc.m210723200 · 2003 · External reference
Regulation of microglial proliferation during chronic neurodegeneration
10.1523/jneurosci.4440-12.2013 · 2013 · External reference
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10.12998/wjcc.v11.i15.3385 · 2023 · External reference
IL-8 and IL-1β in the cerebrospinal fluid of patient with Creutzfeldt-Jakob disease: a case report
10.1002/ccr3.70508 · 2025 · External reference
Doxycycline in Creutzfeldt-Jakob disease: a phase 2, randomised, double-blind, placebo-controlled trial
10.1016/s1474-4422(13)70307-7 · 2014 · External reference
New and distinct chronic wasting disease strains associated with cervid polymorphism at codon 116 of the Prnp gene
10.1371/journal.ppat.1009795 · 2021 · External reference
Complement 3+-astrocytes are highly abundant in prion diseases, but their abolishment led to an accelerated disease course and early dysregulation of microglia
10.1186/s40478-019-0735-1 · 2019 · External reference
10.1371/journal.ppat.1012582 · 2025 · Admitted local publication
Brain biopsy in patients with suspected Creutzfeldt-Jakob disease
10.3171/jns/2008/109/10/0735 · 2008 · External reference
Rapidly progressive dementias - aetiologies, diagnosis and management
10.1038/s41582-022-00659-0 · 2022 · External reference
Prion infections and anti-PrP antibodies trigger converging neurotoxic pathways
10.1371/journal.ppat.1004662 · 2015 · External reference
The same prion strain causes vCJD and BSE
10.1038/38925 · 1997 · External reference
New insights on the role of microglia in synaptic pruning in health and disease
10.1016/j.conb.2015.12.004 · 2016 · External reference
Creutzfeldt-Jakob disease
2017 · External reference
Replacing microglia to treat Alzheimer's disease
10.1016/j.stem.2023.07.005 · 2023 · External reference
Prion diseases
10.1016/s1474-4422(05)70192-7 · 2005 · External reference
A camelid anti-PrP antibody abrogates PrP replication in prion-permissive neuroblastoma cell lines
10.1371/journal.pone.0009804 · 2010 · External reference
Trends and challenges of AAV-delivered gene editing therapeutics for CNS disorders: implications for neurodegenerative disease
10.1016/j.omtn.2025.102635 · 2025 · External reference
Utility of RNAi-mediated prnp gene silencing in neuroblastoma cells permanently infected by prions: potentials and limitations
10.1016/j.antiviral.2009.09.002 · 2009 · External reference
The rise of astrocytes: are they guardians or troublemakers of the brain disorder?
10.1038/s12276-025-01627-6 · 2026 · External reference
Impairment of neuronal mitochondrial quality control in prion-induced neurodegeneration
10.3390/cells11172744 · 2022 · External reference
Genetic PrP prion diseases
10.1101/cshperspect.a033134 · 2018 · External reference
The advances in the early and accurate diagnosis of Creutzfeldt-Jakob disease and other prion diseases: where are we today?
10.1080/14737175.2023.2246653 · 2023 · External reference
PrP antibodies do not trigger mouse hippocampal neuron apoptosis
2012 · External reference
Peripheral administration of a humanized anti-PrP antibody blocks Alzheimer's disease aβ synaptotoxicity
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Clinical diagnosis of human prion disease
10.1016/bs.pmbts.2020.07.006 · 2020 · External reference
Distinct microglia profile in Creutzfeldt-Jakob disease and Alzheimer's disease is independent of disease kinetics
10.1111/neup.12517 · 2018 · External reference
Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner
10.1084/jem.20161547 · 2017 · External reference
Reactive astrocytes associated with prion disease impair the blood brain barrier
10.1016/j.nbd.2023.106264 · 2023 · External reference
Non-cell autonomous astrocyte-mediated neuronal toxicity in prion diseases
10.1186/s40478-021-01123-8 · 2021 · External reference
The N-terminal cleavage of cellular prion protein in the human brain
10.1016/j.febslet.2005.10.013 · 2005 · External reference
Comparison of CR36, a new heparan mimetic, and pentosan polysulfate in the treatment of prion diseases
10.1099/vir.0.82286-0 · 2007 · External reference
Doxycycline rescues recognition memory and circadian motor rhythmicity but does not prevent terminal disease in fatal familial insomnia mice
10.1016/j.nbd.2021.105455 · 2021 · External reference
Supportive care or exhausted neglect: the role of microglia at the end stage of prion disease
10.1172/jci186940 · 2024 · External reference
Prion diseases as transmissible zoonotic diseases
10.1016/j.phrp.2012.12.008 · 2013 · External reference
Systemic delivery of siRNA down regulates brain prion protein and ameliorates neuropathology in prion disorder
10.1371/journal.pone.0088797 · 2014 · External reference
CYP46A1 activation by low-dose efavirenz enhances brain cholesterol metabolism in subjects with early Alzheimer's disease
10.1186/s13195-022-01151-z · 2022 · External reference
The oligomer modulator anle138b inhibits disease progression in a Parkinson mouse model even with treatment started after disease onset
10.1007/s00401-014-1265-3 · 2014 · External reference
Safety, tolerability and pharmacokinetics of the oligomer modulator anle138b with exposure levels sufficient for therapeutic efficacy in a murine Parkinson model: a randomised, double-blind, placebo-controlled phase 1a trial
10.1016/j.ebiom.2022.104021 · 2022 · External reference
Neuroinflammation in prion disease
10.3390/ijms22042196 · 2021 · External reference
Neurotoxic reactive astrocytes are induced by activated microglia
10.1038/nature21029 · 2017 · External reference
Plasma and brain concentrations of doxycycline after single and repeated doses in wild-type and APP23 mice
10.1124/jpet.118.252064 · 2019 · External reference
Long-term efavirenz use is associated with worse neurocognitive functioning in HIV-infected patients
10.1007/s13365-015-0382-7 · 2016 · External reference
Region-specific response of astrocytes to prion infection
10.3389/fnins.2019.01048 · 2019 · External reference
PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology
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Neurons and astrocytes respond to prion infection by inducing microglia recruitment
10.1523/jneurosci.4303-03.2004 · 2004 · External reference
Therapeutic targeting of cellular prion protein: toward the development of dual mechanism anti-prion compounds
10.4103/nrr.nrr-d-24-00181 · 2025 · External reference
Pharmacologic stimulation of cytochrome P450 46A1 and cerebral cholesterol turnover in mice
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Focal sharp waves are a specific early-stage marker of the MM2-cortical form of sporadic Creutzfeldt-Jakob disease
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Prion protein monoclonal antibody (PRN100) therapy for Creutzfeldt-Jakob disease: evaluation of a first-in-human treatment programme
10.1016/s1474-4422(22)00082-5 · 2022 · External reference
Perspectives on CRISPR genome editing to prevent prion diseases in high-risk individuals
10.3390/biomedicines12081725 · 2024 · External reference
CRISPR-Cas9-based knockout of the prion protein and its effect on the proteome
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14-3-3 proteins-an update
10.1038/sj.cr.7290291 · 2005 · External reference
Prion protein lowering is a disease-modifying therapy across prion disease stages, strains and endpoints
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Systematic review of pharmacological management in Creutzfeldt-Jakob disease: no options so far?
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Impaired prion replication in spleens of mice lacking functional follicular dendritic cells
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Glial alterations in human prion diseases: a correlative study of astroglia, reactive microglia, protein deposition, and neuropathological lesions
10.1097/md.0000000000010320 · 2018 · External reference
Species barriers in prion diseases--brief review
10.1007/3-211-29981-5_15 · 2005 · External reference
A single-cell map of antisense oligonucleotide activity in the brain
10.1093/nar/gkad371 · 2023 · External reference
Patient reported central nervous system adverse events of Efavirenz-based antiretroviral therapy in people living with HIV in Northwest Ethiopia
10.2147/hiv.s276111 · 2020 · External reference
Calcineurin inhibition at the clinical phase of prion disease reduces neurodegeneration, improves behavioral alterations and increases animal survival
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A conceptual framework for astrocyte function
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Intracerebral infusion of antisense oligonucleotides into prion-infected mice
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Postmortem findings in a case of variant Creutzfeldt-Jakob disease treated with intraventricular pentosan polysulfate
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The role of microglia in prion diseases: a paradigm of functional diversity
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Therapeutic effect of peripheral administration of an anti-prion protein antibody on mice infected with prions
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Efficacy of flupirtine on cognitive function in patients with CJD: a double-blind study
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CRISPR/cas genome editing for neurodegenerative diseases: mechanisms, therapeutic advances, and clinical prospects
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Molecular pathology of fatal familial insomnia
10.1111/j.1750-3639.1998.tb00176.x · 1998 · External reference
Microglia in prion diseases: angels or demons?
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Antibodies inhibit prion propagation and clear cell cultures of prion infectivity
10.1038/35089090 · 2001 · External reference
Flupirtine partially prevents neuronal injury induced by prion protein fragment and lead acetate
10.1006/neur.1995.0044 · 1995 · External reference
Effect of flupirtine on Bcl-2 and glutathione level in neuronal cells treated in vitro with the prion protein fragment (PrP106-126)
10.1006/exnr.1997.6559 · 1997 · External reference
Anti-PrP antibodies block PrPSc replication in prion-infected cell cultures by accelerating PrPC degradation
10.1111/j.1471-4159.2004.02356.x · 2004 · External reference
The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes
10.1371/journal.pone.0003872 · 2008 · External reference
Microglia biology: one century of evolving concepts
10.1016/j.cell.2019.08.053 · 2019 · External reference
Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie
10.1093/emboj/16.20.6057 · 1997 · External reference
Antisense oligonucleotides extend survival of prion-infected mice
10.1172/jci.insight.131175 · 2019 · External reference
A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody
10.1038/s41598-022-13455-z · 2022 · External reference
Differential toxicity of antibodies to the prion protein
10.1371/journal.ppat.1005401 · 2016 · External reference
Lithium as a disease-modifying agent for prion diseases
10.1038/s41398-018-0209-4 · 2018 · External reference
Differential inhibition of prion propagation by enantiomers of quinacrine
10.1097/01.lab.0000074919.08232.a2 · 2003 · External reference
Application of an in vitro-amplification assay as a novel pre-screening test for compounds inhibiting the aggregation of prion protein scrapie
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10.3390/biomedicines13092167 · 2025 · External reference
Diagnostic performance of CSF biomarkers in a well-characterized Australian cohort of sporadic Creutzfeldt-Jakob disease
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Treatment of a genetic brain disease by CNS-wide microglia replacement
10.1126/scitranslmed.abl9945 · 2022 · External reference
Prion therapeutics: lessons from the past
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Cellular and molecular mechanisms of prion disease
10.1146/annurev-pathmechdis-012418-013109 · 2019 · External reference
Diagnostic performance of cerebrospinal fluid total tau and phosphorylated tau in Creutzfeldt-Jakob disease: results from the Swedish mortality registry
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Bovine spongiform encephalopathy (BSE) and its epidemiology
10.1093/bmb/66.1.185 · 2003 · External reference
Astrocyte unfolded protein response induces a specific reactivity state that causes non-cell-autonomous neuronal degeneration
10.1016/j.neuron.2019.12.014 · 2020 · External reference
Astrocytes: biology and pathology
10.1007/s00401-009-0619-8 · 2010 · External reference
Lithium alleviates neurotoxic prion peptide-induced synaptic damage and neuronal death partially by the upregulation of nuclear target REST and the restoration of Wnt signaling
10.1016/j.neuropharm.2017.05.021 · 2017 · External reference
Interleukin 4 and interleukin 10 levels are elevated in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease
10.1001/archneur.62.10.1591 · 2005 · External reference
Pro- and anti-inflammatory cytokines in the CSF of patients with Creutzfeldt-Jakob disease
10.1016/j.jneuroim.2005.10.008 · 2006 · External reference
Astrocyte in prion disease: a double-edged sword
10.4103/1673-5374.332202 · 2022 · External reference
Pentosan polysulfate induces low-level persistent prion infection keeping measurable seeding activity without PrP-res detection in Fukuoka-1 infected cell cultures
10.1038/s41598-022-12049-z · 2022 · External reference
In vitro neutralization of prions with PrP(Sc)-specific antibodies
10.1080/19336896.2015.1071761 · 2015 · External reference
Pharmacological activation of autophagy favors the clearing of intracellular aggregates of misfolded prion protein peptide to prevent neuronal death
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Doxycycline control of prion protein transgene expression modulates prion disease in mice
10.1073/pnas.95.21.12580 · 1998 · External reference
Continuous intraventricular infusion of pentosan polysulfate: clinical trial against prion diseases
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10.1016/s1473-3099(19)30615-2 · 2020 · External reference
Cerebrospinal fluid and plasma biomarkers in individuals at risk for genetic prion disease
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Pathogenesis of natural scrapie in sheep
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Doxycycline in early CJD: a double-blinded randomised phase II and observational study
10.1136/jnnp-2016-313541 · 2017 · External reference
The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein
2001 · External reference
Anle138b: a novel oligomer modulator for disease-modifying therapy of neurodegenerative diseases such as prion and Parkinson's disease
10.1007/s00401-013-1114-9 · 2013 · External reference
Gene-edited murine cell lines for propagation of chronic wasting disease prions
10.1038/s41598-019-47629-z · 2019 · External reference
PMCA for ultrasensitive detection of prions and to study disease biology
10.1007/s00441-022-03727-5 · 2023 · External reference
Knockdown of prion protein (PrP) by RNA interference weakens the protective activity of wild-type PrP against copper ion and antagonizes the cytotoxicity of fCJD-associated PrP mutants in cultured cells
10.3892/ijmm.2011.688 · 2011 · External reference
The importance of ongoing international surveillance for Creutzfeldt-Jakob disease
10.1038/s41582-021-00488-7 · 2021 · External reference
A naturally occurring C-terminal fragment of the prion protein (PrP) delays disease and acts as a dominant-negative inhibitor of PrPSc formation
10.1074/jbc.m111.286195 · 2011 · External reference
Monoclonal antibodies inhibit prion replication and delay the development of prion disease
10.1038/nature01457 · 2003 · External reference
Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease
10.1073/pnas.0802759105 · 2008 · External reference
Comparison of the anti-prion mechanism of four different anti-prion compounds, anti-PrP monoclonal antibody 44B1, pentosan polysulfate, chlorpromazine, and U18666A, in prion-infected mouse neuroblastoma cells
10.1371/journal.pone.0106516 · 2014 · External reference
A cell therapy approach to restore microglial Trem2 function in a mouse model of Alzheimer's disease
10.1016/j.stem.2023.07.006 · 2023 · External reference
SiRNA therapeutics for protein misfolding diseases of the central nervous system
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Clinical diagnosis and differential diagnosis of CJD and vCJD. With special emphasis on laboratory tests
10.1034/j.1600-0463.2002.100111.x · 2002 · External reference
Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein
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