☆ 4.1 Review

Human prion diseases and the prion protein - what is the current state of knowledge?

TRANSLATIONAL NEUROSCIENCE (2023)

相关参考文献

注意：仅列出部分参考文献，下载原文获取全部文献信息。

Review Cell Biology

Anchorless risk or released benefit? An updated view on the ADAM10-mediated shedding of the prion protein

Behnam Mohammadi et al.

Summary: This review provides a comprehensive overview of the shedding process of the prion protein (PrP) and discusses its role in regulating cellular functions, neurodegenerative diseases, intercellular communication, and the need for improved research tools. Deeper mechanistic insight into PrP shedding and its resulting fragment may lead to improved diagnostics and therapeutic approaches in brain and other diseases.

CELL AND TISSUE RESEARCH (2023)

添加到收藏夹

Article Biochemistry & Molecular Biology

A single protective polymorphism in the prion protein blocks cross-species prion replication in cultured cells

Hamza Arshad et al.

Summary: The bank vole prion protein can act as a universal acceptor of prions from different species. Through a cell culture paradigm, it has been found that bank vole prion protein can facilitate cross-species prion replication, and a single amino acid change can alter its permissiveness to prions. This cellular model will be valuable for studying the molecular features of bank vole prion protein as a universal prion acceptor.

JOURNAL OF NEUROCHEMISTRY (2023)

添加到收藏夹

Review Biochemistry & Molecular Biology

Prion Mutations in Republic of Republic of Korea, China, and Japan

Dan Yeong Kim et al.

Summary: Prion gene mutations are associated with diverse disease phenotypes and have been reported in various neurodegenerative diseases. This review focuses on prion mutations in Asian countries, highlighting the clinical phenotypes and imaging data associated with these mutations. Several prion mutations are specific to Asians and have rarely been reported in other populations. These differences may be attributed to geographical or ethical isolation. Further studies are needed to investigate additional genetic factors that influence disease progression or act as neuroprotective factors.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2023)

添加到收藏夹

Article Medicine, General & Internal

Significance of Cortical Ribboning as a Biomarker in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease

Yasuhiro Hamada et al.

Summary: A case study of a 63-year-old woman with orofacial dystonia showed cortical ribboning on MRI, a typical presentation of sporadic Creutzfeldt-Jakob disease (sCJD). However, the real-time quaking-induced conversion (RT-QuIC) test, the most sensitive method for early diagnosis of sCJD, initially came out negative. Six months later, when the patient was diagnosed with sCJD, the RT-QuIC test became positive. The cerebral blood flow showed a decrease in the cerebral cortex, consistent with cortical ribboning, but an increase in the basal ganglia, possibly related to orofacial dystonia. The study suggests that cortical ribboning on MRI may be a more reliable biomarker than RT-QuIC in the prodromal phase of sCJD.

INTERNAL MEDICINE (2022)

添加到收藏夹

Article Clinical Neurology

Clinical profile of fatal familial insomnia: phenotypic variation in 129 polymorphisms and geographical regions

Jing Zhang et al.

Summary: Insomnia, rapid progressive dementia, and hypertension are the representative key clinical presentations of FFI. Phenotypic variations in genotypes and geographic regions were documented. Prion protein gene 129 Met was considered to be a risk factor for FFI in the non-Asian population, and 129 polymorphisms could modify survival duration.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2022)

添加到收藏夹

Article Biochemistry & Molecular Biology

The first non-prion pathogen identified: neurotropic influenza virus

Suehiro Sakaguchi et al.

Summary: This study reveals that influenza A virus (IAV) can induce the conversion of cellular prion protein (PrP(C)) to the disease-associated isoform (PrPSc) and propagate infectious prions in neuronal cells. This finding suggests a potential link between IAV infection and sporadic prion diseases.

PRION (2022)

添加到收藏夹

Review Hematology

Importation of plasma and use of apheresis platelets as risk reduction measures for variant Creutzfeldt-Jakob disease: The SaBTO review

Stephen Thomas et al.

Summary: This study discusses the review and recommendations on the vCJD risk reduction measures in the UK, suggesting the withdrawal of current measures for individuals born after 1995 or with TTP to save resources and improve supply equality.

TRANSFUSION MEDICINE (2022)

添加到收藏夹

Article Virology

Phenotypic Heterogeneity of Variably Protease-Sensitive Prionopathy: A Report of Three Cases Carrying Different Genotypes at PRNP Codon 129

Simone Baiardi et al.

Summary: Variably protease-sensitive prionopathy is a rare and underestimated prion disease with diverse and often non-specific clinical and pathological features. The host genetic background, particularly the PRNP codon 129 genotype, plays a significant role in phenotypic modulation. The sensitivity of pathological prion protein to proteinase K digestion varies based on different genotypes, leading to distinct PrPSc profiles and cerebral/cerebellar PrP mini-plaque patterns.

VIRUSES-BASEL (2022)

添加到收藏夹

Article Veterinary Sciences

Evaluation of Real-Time Quaking-Induced Conversion, ELISA, and Immunohistochemistry for Chronic Wasting Disease Diagnosis

Carine L. Holz et al.

Summary: Chronic wasting disease (CWD) is a transmissible prion disorder that primarily affects cervids across various regions. Different diagnostic methods such as ELISA, IHC, and RT-QuIC have been compared in a study on white-tailed deer in Michigan, showing that RT-QuIC may be a comparable and useful tool for routine CWD detection. Further multi-laboratory validation is being conducted to assess the variability and sensitivity of these diagnostic methods.

FRONTIERS IN VETERINARY SCIENCE (2022)

添加到收藏夹

Article Medicine, General & Internal

Validation of Revised International Creutzfeldt-Jakob Disease Surveillance Network Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease

Neil Watson et al.

Summary: This diagnostic study demonstrated significantly improved sensitivity of revised sCJD diagnostic criteria with unaltered specificity. The revision has enhanced diagnostic accuracy for clinical care and surveillance.

JAMA NETWORK OPEN (2022)

添加到收藏夹

Article Clinical Neurology

Sporadic Fatal Insomnia Presenting with Initial Symptoms of Parkinsonism and Abnormal Dopamine Transporter Imaging

Tatevik Mkhitarjan et al.

MOVEMENT DISORDERS CLINICAL PRACTICE (2022)

添加到收藏夹

Article Clinical Neurology

Diagnostic accuracy of cerebrospinal fluid biomarkers in genetic prion diseases

Matthias Schmitz et al.

Summary: This study aimed to analyze the diagnostic accuracy of cerebrospinal fluid (CSF) biomarkers used in the clinical diagnosis of genetic prion diseases. The study included 302 symptomatic cases from 11 prion diagnostic centers, encompassing 36 different pathogenic sequence variations. The results showed variations in the accuracy and associations of biomarkers in different genetic prion diseases.

BRAIN (2022)

添加到收藏夹

Article Clinical Neurology

Plasma neurofilament light chain as a biomarker for fatal familial insomnia

Peter Hermann et al.

Summary: Plasma neurofilament light chain may be a valuable diagnostic and prognostic biomarker for fatal familial insomnia.

EUROPEAN JOURNAL OF NEUROLOGY (2022)

添加到收藏夹

Article Clinical Neurology

Proposal of new diagnostic criteria for fatal familial insomnia

Min Chu et al.

Summary: This study proposes new clinical diagnostic criteria for fatal familial insomnia (FFI), aiming to refine the clinical hallmarks of the disease and aid in the early recognition of FFI and differentiation from other prion diseases.

JOURNAL OF NEUROLOGY (2022)

添加到收藏夹

Article Clinical Neurology

Assessing initial MRI reports for suspected CJD patients

Aaron Jesuthasan et al.

Summary: This study reviewed the reporting sensitivity of MRI scans for sporadic Creutzfeldt-Jakob disease (sCJD) cases a decade later. While there has been improvement in local MRI reporting, characteristic abnormalities are still significantly under-detected on initial scans. Sensitivity is higher when the cerebral cortex and multiple regions are involved.

JOURNAL OF NEUROLOGY (2022)

添加到收藏夹

Review Virology

Transport of Prions in the Peripheral Nervous System: Pathways, Cell Types, and Mechanisms

Sam M. Koshy et al.

Summary: Prion diseases are transmissible protein misfolding disorders that cause inflammation, cellular degeneration, and death in the central nervous system. The spread of prions from peripheral sites to the central nervous system occurs through neuroanatomical networks. Current research focuses on the mechanisms of prion transport associated with axons.

VIRUSES-BASEL (2022)

添加到收藏夹

Article Geriatrics & Gerontology

PMCA-Based Detection of Prions in the Olfactory Mucosa of Patients With Sporadic Creutzfeldt-Jakob Disease

Federico Angelo Cazzaniga et al.

Summary: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder caused by the conformational conversion of the prion protein. The PMCA technique can detect PrPSc in the olfactory mucosa and estimate its concentration. Optimizing PMCA is important for diagnosis and selection of patients for clinical trials.

FRONTIERS IN AGING NEUROSCIENCE (2022)

添加到收藏夹

Review Geriatrics & Gerontology

The Use of Real-Time Quaking-Induced Conversion for the Diagnosis of Human Prion Diseases

Anna Poleggi et al.

Summary: Prion diseases are transmissible neurodegenerative disorders that are difficult to diagnose. The ultrasensitive RT-QuIC assay has greatly improved the diagnostic process with its high sensitivity and specificity. It also holds great potential for early and preclinical diagnosis.

FRONTIERS IN AGING NEUROSCIENCE (2022)

添加到收藏夹

Article Biochemistry & Molecular Biology

Carnosic Acid and Carnosol Display Antioxidant and Anti-Prion Properties in In Vitro and Cell-Free Models of Prion Diseases

Korina Karagianni et al.

Summary: The catechol-type diterpene Carnosic acid (CA) and its metabolite Carnosol (CS) from Rosmarinus officinalis have antioxidant and neuroprotective effects, reducing the accumulation and disrupting the aggregation of disease-associated PrP in prion diseases.

ANTIOXIDANTS (2022)

添加到收藏夹

Article Microbiology

Assessment of Real-Time Quaking-Induced Conversion (RT-QuIC) Assay, Immunohistochemistry and ELISA for Detection of Chronic Wasting Disease under Field Conditions in White-Tailed Deer: A Bayesian Approach

Catalina Picasso-Risso et al.

Summary: This study evaluated the accuracy of a new prion amplification assay (RT-QuIC) for diagnosing chronic wasting disease (CWD). The results showed that RT-QuIC performed similarly to ELISA/IHC in detecting CWD in the tonsil and submandibular lymph nodes of wild white-tailed deer. The specificity of RT-QuIC was also high.

PATHOGENS (2022)

添加到收藏夹

Article Clinical Neurology

Cryo-EM structures of prion protein filaments from Gerstmann-Straussler-Scheinker disease

Grace Hallinan et al.

Summary: The aggregation of Prion protein (PrP) and formation of PrP amyloid (APrP) are crucial events in the development of prion diseases. Gerstmann-Straussler-Scheinker (GSS) disease, a dominantly inherited form of prion protein amyloidosis, is characterized by the presence of PrP amyloid plaques throughout the brain. Cryo-EM analysis of APrP filaments isolated from GSS patients with the F198S mutation revealed their complex structure, highlighting the importance of understanding filament structures in human neurodegenerative diseases.

ACTA NEUROPATHOLOGICA (2022)

添加到收藏夹

Review Genetics & Heredity

Genetic counseling for prion disease: Updates and best practices

Jill S. Goldman et al.

Summary: Prion disease is a rare and fatal neurodegenerative disease caused by autosomal dominant variants in the PRNP gene. Diagnosis is complicated by rarity and phenotypic variability, often obscuring family history. Recent developments in prion-detection assay and characterization of PRNP variants have improved symptomatic diagnosis and understanding of the disease. The traditional genotype-phenotype correlation is weakening over time, and the term genetic prion disease may now better serve healthcare providers.

GENETICS IN MEDICINE (2022)

添加到收藏夹

Letter Medicine, General & Internal

A case of rapidly progressive insomnia and dysautonomia

Jingwei Sim et al.

ANNALS ACADEMY OF MEDICINE SINGAPORE (2022)

添加到收藏夹

Article Neuroimaging

Specific structuro-metabolic pattern of thalamic subnuclei in fatal familial insomnia: A PET/MRI imaging study

Kexin Xie et al.

Summary: The study revealed a specific structuro-metabolic pattern of fatal familial insomnia, highlighting the essential roles of the medial dorsal nuclei, anterior nuclei, and pulvinar as potential biomarkers for diagnosis. In addition, primary thalamic subnuclei alterations may be correlated with insomnia, neuropsychiatric, and autonomic symptoms, without primary cortical involvement.

NEUROIMAGE-CLINICAL (2022)

添加到收藏夹

Article Veterinary Sciences

No evidence of uptake or propagation of reindeer CWD prions in environmentally exposed sheep

Erez Harpaz et al.

Summary: This study investigates the spatial and time-relevant overlaps between CWD-infected reindeer and sheep using GPS data. The results show an overlap in area use between the two species, but no prions were detected in the GALT of the investigated sheep.

ACTA VETERINARIA SCANDINAVICA (2022)

添加到收藏夹

Review Medicine, General & Internal

Role of Biomarkers for the Diagnosis of Prion Diseases: A Narrative Review

Miren Altuna et al.

Summary: Prion diseases are progressive neurodegenerative disorders with varied symptoms, including rapid dementia, cerebellar ataxia, and myoclonus. Diagnostic criteria and biomarkers have been developed, but early diagnosis remains challenging. Researchers are exploring more efficient biomarkers for early detection.

MEDICINA-LITHUANIA (2022)

添加到收藏夹

Article Cell Biology

NMDA Receptor and L-Type Calcium Channel Modulate Prion Formation

Marco Zattoni et al.

Summary: This study demonstrates that changes in LTCCs and NMDARs activities can modulate PrPSc formation through ERK signaling, suggesting a potential link between synaptic plasticity pathways and prion conversion. Contrasting intracellular signals during synaptic plasticity may influence time-dependent prion conversion.

CELLULAR AND MOLECULAR NEUROBIOLOGY (2021)

添加到收藏夹

Letter Clinical Neurology

E200K familial Creutzfeldt-Jakob disease. MRI, EEG, PET and neuropathological correlation in a family

A. Sanchez-Soblechero et al.

NEUROLOGIA (2021)

添加到收藏夹

Article Biochemistry & Molecular Biology

Extracellular Prion Protein Aggregates in Nine Gerstmann-Straussler-Scheinker Syndrome Subjects with Mutation P102L: A Micromorphological Study and Comparison with Literature Data

Nikol Jankovska et al.

Summary: GSS is a hereditary neurodegenerative disease characterized by PrP forming pathological plaques. Most GSS plaques are small, condensed, and do not contain other pathological protein components, with the presence of dystrophic neuritic structures being rare.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

添加到收藏夹

Review Biochemistry & Molecular Biology

Virus Infection, Genetic Mutations, and Prion Infection in Prion Protein Conversion

Hideyuki Hara et al.

Summary: The article discusses the mechanism of PrP(C) conversion into PrPSc in different types of prion diseases, involving sporadic, hereditary, and acquired disorders, as well as the pathogenic role of neurotropic influenza A virus (IAV) in sporadic prion diseases.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

添加到收藏夹

Review Biochemistry & Molecular Biology

Neuroinflammation in Prion Disease

Bei Li et al.

Summary: Neuroinflammation, characterized by microglial activation and astrogliosis, is a common feature in neurodegenerative conditions like prion diseases. Microglia have a neuroprotective role, while reactive astrocytes can propagate prions and contribute to neurodegeneration. Targeting neuroinflammation could be a novel approach for treating prion diseases and understanding other neurodegenerative disorders.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

添加到收藏夹

Review Biochemistry & Molecular Biology

Neurometals in the Pathogenesis of Prion Diseases

Masahiro Kawahara et al.

Summary: Prion diseases are progressive and transmissive neurodegenerative diseases, where the conversion of normal cellular prion protein (PrP(C)) into abnormal pathogenic prion protein (PrPSc) is critical. PrP(C) has the ability to bind to various neurometals and plays essential roles in maintaining the homeostasis of these neurometals at the synapse, which are critical determinants of the conformational change and toxicity of PrP(C).

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

添加到收藏夹

Review Clinical Neurology

Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease

Peter Hermann et al.

Summary: Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins. Its diagnosis relies on a combination of neuropsychiatric symptoms, CSF markers, and imaging. The development of assays like RT-QuIC has improved diagnostic accuracy.

LANCET NEUROLOGY (2021)

添加到收藏夹

Article Clinical Neurology

Genetic Creutzfeldt-Jakob disease-M232R with the cooccurrence of multiple prion strains, M1+M2C+M2T: Report of an autopsy case

Masayuki Shintaku et al.

Summary: The translation describes a rare case of gCJD with M232R mutation in Japan, with the patient presenting symptoms of dementia and sleep disturbance. Autopsy results showed widespread spongiform changes in the cerebral neocortex, predominantly synaptic type PrPSc deposition, and the presence of M2T strain.

NEUROPATHOLOGY (2021)

添加到收藏夹

Review Clinical Neurology

Environmental and host factors that contribute to prion strain evolution

Jason C. Bartz

Summary: Prions are novel pathogens composed of PrPSc, with strain distribution influenced by both environmental and host factors. The host amino acid sequence and post-translational modifications of PrP(C) play a critical role in dictating the repertoire of prion strains, and interference between prion strains may impact the emergence of dominant strains.

ACTA NEUROPATHOLOGICA (2021)

添加到收藏夹

Review Microbiology

Variant CJD: Reflections a Quarter of a Century on

Diane L. Ritchie et al.

Summary: Twenty-five years after variant Creutzfeldt-Jakob disease (vCJD) was first described in the UK, extensive experimental evidence confirms vCJD as a zoonotic disease caused by dietary exposure to the BSE agent. Despite low numbers of vCJD cases, concerns persist over the emergence of new cases in other genetic cohorts and the presence of asymptomatic individuals carrying vCJD infectivity in the population.

PATHOGENS (2021)

添加到收藏夹

Review Virology

Clinical Use of Improved Diagnostic Testing for Detection of Prion Disease

Mark P. Figgie et al.

Summary: Prion diseases are challenging to diagnose early on, with symptoms typically not fully manifesting until late in the disease course. The introduction of second-generation RT-QuIC technology has significantly improved pre-mortem diagnosis of prion disease, but its efficacy may be limited in rarer and atypical cases.

VIRUSES-BASEL (2021)

添加到收藏夹

Article Clinical Neurology

Selective vulnerability to atrophy in sporadic Creutzfeldt-Jakob disease

Kyan Younes et al.

Summary: Brain atrophy in sporadic Creutzfeldt-Jakob disease (sCJD) selectively affects specific cortical and subcortical regions, with involvement of regions overlapping with the default mode network (DMN) and insulae, thalami, and right occipital lobe. Structural equation modeling (SEM) shows an effective connectivity model in sCJD, but not in controls. Presence of visual hallucinations correlates with atrophy in specific brain regions.

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY (2021)

添加到收藏夹

Article Microbiology

Further Characterization of Glycoform-Selective Prions of Variably Protease-Sensitive Prionopathy

Weiguanliu Zhang et al.

Summary: This study reveals the formation process of ladder-like PrPSc in variably protease-sensitive prionopathy (VPSPr), emphasizing its association with basic pH conditions. The research findings suggest that the glycoform-selective PrPSc in VPSPr is characterized by altered glycosylation and exhibits lower aggregation seeding activity compared to PrPSc in sporadic CJD (sCJD).

PATHOGENS (2021)

添加到收藏夹

Article Clinical Neurology

Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification

Simone Baiardi et al.

Summary: The current classification of sporadic Creutzfeldt-Jakob disease (sCJD) includes six major clinicopathological subtypes based on the physicochemical properties of the protease-resistant core of the pathologic prion protein (PrPSc), defining two major PrPSc types (1 and 2), and the codon 129 genotype on the prion protein gene (PRNP). The research analyzed 208 individuals affected by genetic CJD (gCJD), identifying six major groups based on the combination of PrPSc type and codon 129 genotype on PRNP mutated allele. The phenotypic variability of gCJD is mostly consistent with that previously found in sCJD.

ACTA NEUROPATHOLOGICA (2021)

添加到收藏夹

Article Clinical Neurology

Structure of Tau filaments in Prion protein amyloidoses

Grace I. Hallinan et al.

Summary: The study analyzes Tau fibers from individuals affected by Prion-Protein cerebral amyloid angiopathy (PrP-CAA) and Gerstmann-Straussler-Scheinker (GSS) disease, showing a striking similarity to neurofibrillary tangles (NFTs) in Alzheimer's disease. Regardless of the primary amino acid sequence of the amyloid protein, similar molecular mechanisms are at play in the formation of identical Tau filaments, as supported by mass spectrometry and cryo-EM analyses.

ACTA NEUROPATHOLOGICA (2021)

添加到收藏夹

Article Cell Biology

Prion protein and prion disease at a glance

Caihong Zhu et al.

Summary: Prion diseases are neurodegenerative disorders caused by the abnormal conformational conversion of cellular prion protein. The phenomenon of prion-like spread has also been observed in other disease-associated proteins, such as Aβ, tau, and α-synuclein. Prion protein may play a role in the pathogenesis of a broad spectrum of neurodegenerative conditions, including Alzheimer's and Parkinson's disease.

JOURNAL OF CELL SCIENCE (2021)

添加到收藏夹

Article Biochemistry & Molecular Biology

High-resolution structure and strain comparison of infectious mammalian prions

Allison Kraus et al.

Summary: The study reveals the core structure of prions and the templating surface formed by individual monomers at fibril ends. Comparison to another prion strain shows differences in fibril morphology. These findings provide insights into the propagation of prions.

MOLECULAR CELL (2021)

添加到收藏夹

Review Clinical Neurology

Sporadic Creutzfeldt-Jakob Disease Appears to Be Sporadic Fatal Insomnia: A Case Report and Review of the Literature

Qingqing Sun et al.

Summary: Creutzfeldt-Jakob disease (CJD) subtypes are challenging to identify due to clinical heterogeneity, with accurate early recognition aiding prognosis prediction. Diagnosis of sCJD subtypes currently relies on brain tissue biopsy or autopsy. This report presents a case of sCJD initially presenting as insomnia, emphasizing sporadic fatal insomnia as a differential diagnosis of sCJD.

NATURE AND SCIENCE OF SLEEP (2021)

添加到收藏夹

Review Veterinary Sciences

Chronic wasting disease in Europe: new strains on the horizon

Michael Andreas Tranulis et al.

Summary: Prion diseases are fatal neurodegenerative disorders caused by misfolded prions, which can be transmitted directly or indirectly. CWD cases have been found in deer and elk, and have emerged in Norway and other Nordic countries, posing a threat to European cervid populations.

ACTA VETERINARIA SCANDINAVICA (2021)

添加到收藏夹

Review Biochemistry & Molecular Biology

The role of microglia in prion diseases and possible therapeutic targets: a literature review

Ananda Sampaio Lamenha Falcao de Melo et al.

Summary: Creutzfeldt-Jakob disease (CJD) is a rare and fatal condition involving progressive neurodegeneration, with a focus on the role of microglia in immune modulation. Microglia activation and phenotype modulation are seen as key in developing new treatment approaches, guiding these cells from harmful to beneficial roles in neuroinflammation.

PRION (2021)

添加到收藏夹

Article Medicine, Research & Experimental

Prion protein glycans reduce intracerebral fibril formation and spongiosis in prion disease

Alejandro M. Sevillano et al.

JOURNAL OF CLINICAL INVESTIGATION (2020)

添加到收藏夹

Article Biochemistry & Molecular Biology

Diagnostic Accuracy of Prion Disease Biomarkers in Iatrogenic Creutzfeldt-Jakob Disease

Franc Llorens et al.

BIOMOLECULES (2020)

添加到收藏夹

Review Biochemistry & Molecular Biology

PMCA Applications for Prion Detection in Peripheral Tissues of Patients with Variant Creutzfeldt-Jakob Disease

Giorgio Giaccone et al.

BIOMOLECULES (2020)

添加到收藏夹

Article Clinical Neurology

Prevalence in Britain of abnormal prion protein in human appendices before and after exposure to the cattle BSE epizootic

O. Noel Gill et al.

ACTA NEUROPATHOLOGICA (2020)

添加到收藏夹

Review Biochemistry & Molecular Biology

The PERK-Dependent Molecular Mechanisms as a Novel Therapeutic Target for Neurodegenerative Diseases

Wioletta Rozpedek-Kaminska et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

添加到收藏夹

Article Clinical Neurology

Detection of prions in skin punch biopsies of Creutzfeldt-Jakob disease patients

Angela Mammana et al.

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY (2020)

添加到收藏夹

Review Microbiology

Immunotherapy against Prion Disease

Yue Ma et al.

PATHOGENS (2020)

添加到收藏夹

Article Clinical Neurology

Transmission of CJD from nasal brushings but not spinal fluid or RT-QuIC product

Gregory J. Raymond et al.

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY (2020)

添加到收藏夹

Article Biochemistry & Molecular Biology

A soluble derivative of PrP©activates cell-signaling and regulates cell physiology through LRP1 and the NMDA receptor

Elisabetta Mantuano et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2020)

添加到收藏夹

Letter Medicine, General & Internal

Variant Creutzfeldt-Jakob Disease Diagnosed 7.5 Years after Occupational Exposure

Jean-Philippe Brandel et al.

NEW ENGLAND JOURNAL OF MEDICINE (2020)

添加到收藏夹

Article Clinical Neurology

Comparison between plasma and cerebrospinal fluid biomarkers for the early diagnosis and association with survival in prion disease

Samir Abu-Rumeileh et al.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2020)

添加到收藏夹

Review Biochemistry & Molecular Biology

Copper Dyshomeostasis in Neurodegenerative Diseases-Therapeutic Implications

Grazyna Gromadzka et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

添加到收藏夹

Review Virology

Exposure Risk of Chronic Wasting Disease in Humans

Satish K. Nemani et al.

VIRUSES-BASEL (2020)

添加到收藏夹

Article Biotechnology & Applied Microbiology

Optimization of the Real-Time Quaking-Induced Conversion Assay for Prion Disease Diagnosis

Inga Zerr et al.

FRONTIERS IN BIOENGINEERING AND BIOTECHNOLOGY (2020)

添加到收藏夹

Article Biochemistry & Molecular Biology

Recent developments in antibody therapeutics against prion disease

Karl Frontzek et al.

EMERGING TOPICS IN LIFE SCIENCES (2020)

添加到收藏夹

Review Ophthalmology

A Contemporary Risk Analysis of Iatrogenic Transmission of Creutzfeldt-Jakob Disease (CJD) via Corneal Transplantation in the United States

Tanisha Martheswaran et al.

OPHTHALMOLOGY AND THERAPY (2020)

添加到收藏夹

Review Biochemistry & Molecular Biology

Function of Prion Protein and the Family Member, Shadoo

Takashi Onodera et al.

CURRENT ISSUES IN MOLECULAR BIOLOGY (2020)

添加到收藏夹

Editorial Material Clinical Neurology

Cortical and bithalamic hypometabolism by FDG-PET/CT in a patient with sporadic fatal insomnia

Taylor Haight et al.

NEUROLOGY (2019)

添加到收藏夹

Review Virology

Kuru, the First Human Prion Disease

Pawel P. Liberski et al.

VIRUSES-BASEL (2019)

添加到收藏夹

Review Virology

Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans

Marcello Rossi et al.

VIRUSES-BASEL (2019)

添加到收藏夹

Review Infectious Diseases

Human prion diseases

Han Wang et al.

CURRENT OPINION IN INFECTIOUS DISEASES (2019)

添加到收藏夹

Article Clinical Neurology

Age at onset in genetic prion disease and the design of preventive clinical trials

Eric Vallabh Minikel et al.

NEUROLOGY (2019)

添加到收藏夹

Article Microbiology

Full atomistic model of prion structure and conversion

Giovanni Spagnolli et al.

PLOS PATHOGENS (2019)

添加到收藏夹

Review Neurosciences

Recent Advances in Understanding Mammalian Prion Structure: A Mini Review

Cassandra Terry et al.

FRONTIERS IN MOLECULAR NEUROSCIENCE (2019)

添加到收藏夹

Review Cell Biology

Structural Consequences of Copper Binding to the Prion Protein

Giulia Salzano et al.

CELLS (2019)

添加到收藏夹

Article Clinical Neurology

Clinical Variability in P102L Gerstmann-Straussler-Scheinker Syndrome

Adam Tesar et al.

ANNALS OF NEUROLOGY (2019)

添加到收藏夹

Review Clinical Neurology

Prion Disease

Kelly J. Baldwin et al.

SEMINARS IN NEUROLOGY (2019)

添加到收藏夹

Review Biochemistry & Molecular Biology

Transition of the prion protein from a structured cellular form (PrP©) to the infectious scrapie agent (PrPSc)

Pravas K. Baral et al.

PROTEIN SCIENCE (2019)

添加到收藏夹

Article Clinical Neurology

Prion neurotoxicity

Nhat T. T. Le et al.

BRAIN PATHOLOGY (2019)

添加到收藏夹

Article Clinical Neurology

Recent advances in the histo-molecular pathology of human prion disease

Simone Baiardi et al.

BRAIN PATHOLOGY (2019)

添加到收藏夹

Review Genetics & Heredity

Genetic Factors in Mammalian Prion Diseases

Simon Mead et al.

ANNUAL REVIEW OF GENETICS, VOL 53 (2019)

添加到收藏夹

Editorial Material Clinical Neurology

Pearls & Oy-sters: Challenging diagnosis of Gerstmann-Straussler-Scheinker disease Clinical and imaging findings

Min Ju Kang et al.

NEUROLOGY (2019)

添加到收藏夹

Article Infectious Diseases

Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation

Lesley Uttley et al.

LANCET INFECTIOUS DISEASES (2019)

添加到收藏夹

Review Clinical Neurology

Variably protease-sensitive prionopathy A differential diagnostic consideration for dementia

Stella H. Kim et al.

NEUROLOGY-CLINICAL PRACTICE (2019)

添加到收藏夹

Article Clinical Neurology

Two distinct prions in fatal familial insomnia and its sporadic form

Atsuko Takeuchi et al.

BRAIN COMMUNICATIONS (2019)

添加到收藏夹

Article Immunology

Variable Protease-Sensitive Prionopathy Transmission to Bank Voles

Romolo Nonno et al.

EMERGING INFECTIOUS DISEASES (2019)

添加到收藏夹

Article Medicine, General & Internal

Gerstmann-Straussler-Scheinker syndrome misdiagnosed as conversion disorder

Aiyang Allen Jiang et al.

BMJ CASE REPORTS (2019)

添加到收藏夹

Article Biochemistry & Molecular Biology

Prion protein stabilizes amyloid-β (Aβ) oligomers and enhances Aβ neurotoxicity in a Drosophila model of Alzheimer's disease

Nadine D. Younan et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2018)

添加到收藏夹

Review Pharmacology & Pharmacy

Toward Therapy of Human Prion Diseases

Adriano Aguzzi et al.

ANNUAL REVIEW OF PHARMACOLOGY AND TOXICOLOGY, VOL 58 (2018)

添加到收藏夹

Article Clinical Neurology

Correlation between clinical and radiologic features of patients with Gerstmann-Straussler-Scheinker syndrome (Pro102Leu)

Michiyoshi Yoshimura et al.

JOURNAL OF THE NEUROLOGICAL SCIENCES (2018)

添加到收藏夹

Review Biochemistry & Molecular Biology

Exosomes and their role in the intercellular trafficking of normal and disease associated prion proteins

Lesley Cheng et al.

MOLECULAR ASPECTS OF MEDICINE (2018)

添加到收藏夹

Article Neurosciences

Structural and mechanistic aspects influencing the ADAM10-mediated shedding of the prion protein

Luise Linsenmeier et al.

MOLECULAR NEURODEGENERATION (2018)

添加到收藏夹

Article Clinical Neurology

Two novel PRNP truncating mutations broaden the spectrum of prion amyloidosis

Sabina Capellari et al.

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY (2018)

添加到收藏夹

Article Clinical Neurology

Sporadic Fatal Insomnia in Europe: Phenotypic Features and Diagnostic Challenges

Samir Abu-Rumeileh et al.

ANNALS OF NEUROLOGY (2018)

添加到收藏夹

Article Microbiology

Prions activate a p38 MAPK synaptotoxic signaling pathway

Cheng Fang et al.

PLOS PATHOGENS (2018)

添加到收藏夹

Article Pathology

Cellular and Molecular Mechanisms of Prion Disease

Christina J. Sigurdson et al.

Annual Review of Pathology-Mechanisms of Disease (2018)

添加到收藏夹

Article Pathology

Rapid amplification of prions from variant Creutzfeldt-Jakob disease cerebrospinal fluid

Marcelo A. Barria et al.

JOURNAL OF PATHOLOGY CLINICAL RESEARCH (2018)

添加到收藏夹

Review Clinical Neurology

The function of the cellular prion protein in health and disease

Joel C. Watts et al.

ACTA NEUROPATHOLOGICA (2018)

添加到收藏夹

Review Clinical Neurology

Characterization of mutations in PRNP (prion) gene and their possible roles in neurodegenerative diseases

Eva Bagyinszky et al.

NEUROPSYCHIATRIC DISEASE AND TREATMENT (2018)

添加到收藏夹

Article Medicine, Research & Experimental

Sporadic and Infectious Human Prion Diseases

Robert G. Will et al.

COLD SPRING HARBOR PERSPECTIVES IN MEDICINE (2017)

添加到收藏夹

Article Biochemistry & Molecular Biology

Cellular prion protein targets amyloid-β fibril ends via its C-terminal domain to prevent elongation

Erin Bove-Fenderson et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2017)

添加到收藏夹

Review Medicine, Research & Experimental

Microglia in prion diseases

Adriano Aguzzi et al.

JOURNAL OF CLINICAL INVESTIGATION (2017)

添加到收藏夹

Letter Medicine, General & Internal

Variant Creutzfeldt-Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129

Tzehow Mok et al.

NEW ENGLAND JOURNAL OF MEDICINE (2017)

添加到收藏夹

Article Multidisciplinary Sciences

Cellular prion protein is present in mitochondria of healthy mice

Robert Faris et al.

SCIENTIFIC REPORTS (2017)

添加到收藏夹

Article Clinical Neurology

Diagnosis of Human Prion Disease Using Real-Time Quaking-Induced Conversion Testing of Olfactory Mucosa and Cerebrospinal Fluid Samples

Matilde Bongianni et al.

JAMA NEUROLOGY (2017)

添加到收藏夹

Review Clinical Neurology

Fatal familial insomnia with abnormal signals on routine MRI: a case report and literature review

Tingting Lu et al.

BMC NEUROLOGY (2017)

添加到收藏夹

Review Clinical Neurology

Prion disease: experimental models and reality

Sebastian Brandner et al.

ACTA NEUROPATHOLOGICA (2017)

添加到收藏夹

Review Biochemistry & Molecular Biology

Physiological Functions of the Cellular Prion Protein

Andrew R. Castle et al.

FRONTIERS IN MOLECULAR BIOSCIENCES (2017)

添加到收藏夹

Article Clinical Neurology

'PrP systemic deposition disease': clinical and pathological characteristics of novel familial prion disease with 2-bp deletion in codon 178

K. Matsuzono et al.

EUROPEAN JOURNAL OF NEUROLOGY (2016)

添加到收藏夹

Article Biochemistry & Molecular Biology

Expression of the Prion Protein Family Member Shadoo Causes Drug Hypersensitivity That Is Diminished by the Coexpression of the Wild Type Prion Protein

Antal Nyeste et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2016)

添加到收藏夹

Article Cell Biology

Regulation of human cerebrospinal fluid malate dehydrogenase 1 in sporadic Creutzfeldt-Jakob disease patients

Matthias Schmitz et al.

AGING-US (2016)

添加到收藏夹

Review Microbiology

Prions: Beyond a Single Protein

Alvin S. Das et al.

CLINICAL MICROBIOLOGY REVIEWS (2016)

添加到收藏夹

Article Multidisciplinary Sciences

The prion protein is an agonistic ligand of the G protein-coupled receptor Adgrg6

Alexander Kuffer et al.

NATURE (2016)

添加到收藏夹

Article Clinical Neurology

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

David J. Bonda et al.

NEUROSURGICAL FOCUS (2016)

添加到收藏夹

Article Biochemistry & Molecular Biology

Familial Creutzfeldt-Jakob Disease: Case report and role of genetic counseling in post mortem testing

Kristin Clift et al.

PRION (2016)

添加到收藏夹

Article Microbiology

The Structural Architecture of an Infectious Mammalian Prion Using Electron Cryomicroscopy

Ester Vazquez-Fernandez et al.

PLOS PATHOGENS (2016)

添加到收藏夹

Review Immunology

Prion diseases: immunotargets and therapy

Jennifer T. Burchell et al.

IMMUNOTARGETS AND THERAPY (2016)

添加到收藏夹

Article Clinical Neurology

Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years

Peter Rudge et al.

BRAIN (2015)

添加到收藏夹

Article Biochemistry & Molecular Biology

Prion protein regulates glutathione metabolism and neural glutamate and cysteine uptake via excitatory amino acid transporter 3

Kathrin Guitart et al.

JOURNAL OF NEUROCHEMISTRY (2015)

添加到收藏夹

Letter Clinical Neurology

A case of variably protease-sensitive prionopathy treated with doxycyclin

Hamid Assar et al.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2015)

添加到收藏夹

Article Cell Biology

The cellular prion protein PrPc is a partner of the Wnt pathway in intestinal epithelial cells

Laura S. Besnier et al.

MOLECULAR BIOLOGY OF THE CELL (2015)

添加到收藏夹

Article Biochemistry & Molecular Biology

Prion aggregates transfer through tunneling nanotubes in endocytic vesicles

Seng Zhu et al.

PRION (2015)

添加到收藏夹

Article Biochemistry & Molecular Biology

Shedding light on prion disease

Markus Glatzel et al.

PRION (2015)

添加到收藏夹

Article Virology

Subcellular distribution of the prion protein in sickness and in health

Susan F. Godsave et al.

VIRUS RESEARCH (2015)

添加到收藏夹

Article Biochemistry & Molecular Biology

The prion protein is critical for DNA repair and cell survival after genotoxic stress

Anne Bravard et al.

NUCLEIC ACIDS RESEARCH (2015)

添加到收藏夹

Article Clinical Neurology

Variably Protease-sensitive Prionopathy in an Apparent Cognitively Normal 93-Year-Old

Nupur Ghoshal et al.

ALZHEIMER DISEASE & ASSOCIATED DISORDERS (2014)

添加到收藏夹

Article Biophysics

Structural and Dynamic Properties of the Human Prion Protein

Wei Chen et al.

BIOPHYSICAL JOURNAL (2014)

添加到收藏夹

Article Clinical Neurology

Postmortem findings in a case of variant Creutzfeldt-Jakob disease treated with intraventricular pentosan polysulfate

P. K. Newman et al.

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2014)

添加到收藏夹

Article Neurosciences

Peripheral Administration of a Humanized Anti-PrP Antibody Blocks Alzheimer's Disease Aβ Synaptotoxicity

Igor Klyubin et al.

JOURNAL OF NEUROSCIENCE (2014)

添加到收藏夹

Article Public, Environmental & Occupational Health

Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005-2011

K. M. Olszowy et al.

PUBLIC HEALTH (2014)

添加到收藏夹

Review Virology

The Structure of Human Prions: From Biology to Structural Models - Considerations and Pitfalls

Claudia Y. Acevedo-Morantes et al.

VIRUSES-BASEL (2014)

添加到收藏夹

Editorial Material Clinical Neurology

PERK: a novel therapeutic target for neurodegenerative diseases?

Tao Ma et al.

ALZHEIMERS RESEARCH & THERAPY (2014)

添加到收藏夹

Article Biochemistry & Molecular Biology

The PrPC Cl fragment derived from the ovine A136R154R171 PRNP allele is highly abundant in sheep brain and inhibits fibrillisation of full-length PrPC protein in vitro

Lauren Campbell et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2013)

添加到收藏夹

Article Cell Biology

Genetics of prion diseases

Sarah E. Lloyd et al.

CURRENT OPINION IN GENETICS & DEVELOPMENT (2013)

添加到收藏夹

Article Biochemistry & Molecular Biology

High levels of Cellular Prion Protein improve astrocyte development

Camila Arantes Hartmann et al.

FEBS LETTERS (2013)

添加到收藏夹

Article Clinical Neurology

The association between the methionine/valine (M/V) polymorphism (rs1799990) in the PRNP gene and the risk of Alzheimer disease: An update by meta-analysis

Jie He et al.

JOURNAL OF THE NEUROLOGICAL SCIENCES (2013)

添加到收藏夹

Article Multidisciplinary Sciences

Epidemiological mechanisms of genetic resistance to kuru

Katherine E. Atkins et al.

JOURNAL OF THE ROYAL SOCIETY INTERFACE (2013)

添加到收藏夹

Article Biochemistry & Molecular Biology

Genotype-phenotype analysis in inherited prion disease with eight octapeptide repeat insertional mutation

Martin Paucar et al.

PRION (2013)

添加到收藏夹

Article Cell Biology

Oral Treatment Targeting the Unfolded Protein Response Prevents Neurodegeneration and Clinical Disease in Prion-Infected Mice

Julie A. Moreno et al.

SCIENCE TRANSLATIONAL MEDICINE (2013)

添加到收藏夹

Article Biochemistry & Molecular Biology

The association between prion proteins and Aβ1-42 oligomers in cytotoxicity and apoptosis

Jae Wook Hyeon et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2012)

添加到收藏夹

Letter Clinical Neurology

Prion protein gene M232R variation is probably an uncommon polymorphism rather than a pathogenic mutation

Jon Beck et al.

BRAIN (2012)

添加到收藏夹

Review Radiology, Nuclear Medicine & Medical Imaging

Imaging of prion diseases

Laurent Letourneau-Guillon et al.

JOURNAL OF MAGNETIC RESONANCE IMAGING (2012)

添加到收藏夹

Article Neurosciences

The Complex PrPc-Fyn Couples Human Oligomeric Aβ with Pathological Tau Changes in Alzheimer's Disease

Megan Larson et al.

JOURNAL OF NEUROSCIENCE (2012)

添加到收藏夹

Article Biochemical Research Methods

Protein misfolding cyclic amplification of infectious prions

Rodrigo Morales et al.

NATURE PROTOCOLS (2012)

添加到收藏夹

Article Multidisciplinary Sciences

Role of the Cellular Prion Protein in Oligodendrocyte Precursor Cell Proliferation and Differentiation in the Developing and Adult Mouse CNS

Ana Bribian et al.

PLOS ONE (2012)

添加到收藏夹

Article Immunology

latrogenic Creutzfeldt-Jakob Disease, Final Assessment

Paul Brown et al.

EMERGING INFECTIOUS DISEASES (2012)

添加到收藏夹

Article Clinical Neurology

A novel seven-octapeptide repeat insertion in the prion protein gene (PRNP) in a Dutch pedigree with Gerstmann-Straussler-Scheinker disease phenotype: comparison with similar cases from the literature

Casper Jansen et al.

ACTA NEUROPATHOLOGICA (2011)

添加到收藏夹

Article Biochemistry & Molecular Biology

Tissue- and cell type-specific modification of prion protein (PrP)-like protein Doppel, which affects PrP endoproteolysis

Akikazu Sakudo et al.

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2011)

添加到收藏夹

Article Biochemistry & Molecular Biology

Common Structural Traits across Pathogenic Mutants of the Human Prion Protein and Their Implications for Familial Prion Diseases

Giulia Rossetti et al.

JOURNAL OF MOLECULAR BIOLOGY (2011)

添加到收藏夹

Review Microbiology

De novo generation of prion strains

David W. Colby et al.

NATURE REVIEWS MICROBIOLOGY (2011)

添加到收藏夹

Article Microbiology

Th2-polarised PrP-specific Transgenic T-cells Confer Partial Protection against Murine Scrapie

Saci Iken et al.

PLOS PATHOGENS (2011)

添加到收藏夹

Article Clinical Neurology

Variably Protease-Sensitive Prionopathy: A New Sporadic Disease of the Prion Protein

Wen-Quan Zou et al.

ANNALS OF NEUROLOGY (2010)

添加到收藏夹

Article Geriatrics & Gerontology

D178N, 129Val and N171S, 129Val Genotype in a Family with Creutzfeldt-Jakob Disease

Brian S. Appleby et al.

DEMENTIA AND GERIATRIC COGNITIVE DISORDERS (2010)

添加到收藏夹

Article Genetics & Heredity

PRNP Allelic Series From 19 Years of Prion Protein Gene Sequencing at the MRC Prion Unit

Jon A. Beck et al.

HUMAN MUTATION (2010)

添加到收藏夹

Article Clinical Neurology

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

I. Zerr et al.

BRAIN (2009)

添加到收藏夹

Article Biochemistry & Molecular Biology

Role of ADAMs in the Ectodomain Shedding and Conformational Conversion of the Prion Protein

David R. Taylor et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2009)

添加到收藏夹

Article Immunology

Prion Protein Expression and Release by Mast Cells After Activation

D. James Haddon et al.

JOURNAL OF INFECTIOUS DISEASES (2009)

添加到收藏夹

Article Multidisciplinary Sciences

Cellular prion protein mediates impairment of synaptic plasticity by amyloid-β oligomers

Juha Lauren et al.

NATURE (2009)

添加到收藏夹

Article Medicine, General & Internal

A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure

Simon Mead et al.

NEW ENGLAND JOURNAL OF MEDICINE (2009)

添加到收藏夹

Article Biochemistry & Molecular Biology

Quantitative and qualitative analysis of cellular prion protein (PrPC) expression in bovine somatic tissues

Oscar A. Peralta et al.

PRION (2009)

添加到收藏夹

Article Clinical Neurology

A novel human disease with abnormal prion protein sensitive to protease

Pierluigi Gambetti et al.

ANNALS OF NEUROLOGY (2008)

添加到收藏夹

Article Biology

The epidemiology of kuru: monitoring the epidemic from its peak to its end

Michael P. Alpers

PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES (2008)

添加到收藏夹

Article Biology

Genetic susceptibility, evolution and the kuru epidemic

Simon Mead et al.

PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES (2008)

添加到收藏夹

Article Biochemistry & Molecular Biology

The CNS glycoprotein Shadoo has PrPC-like protective properties and displays reduced levels in prion infections

Joel C. Watts et al.

EMBO JOURNAL (2007)

添加到收藏夹

Article Clinical Neurology

Novel locus for benign hereditary chorea with adult onset maps to chromosome 8q21.3-q23.3

Takayoshi Shimohata et al.

BRAIN (2007)

添加到收藏夹

Article Neurosciences

Contributions of neuronal prion protein on sleep recovery and stress response following sleep deprivation

Manuel Sanchez-Alavez et al.

BRAIN RESEARCH (2007)

添加到收藏夹

Review Biochemistry & Molecular Biology

The prion protein family: Diversity, rivalry, and dysfunction

Joel C. Watts et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2007)

添加到收藏夹

Article Medicine, General & Internal

Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report

Stephen J. Wroe et al.

LANCET (2006)

添加到收藏夹

Article Clinical Neurology

Immunohistochemical expression of prion protein (PrPC) in the human forebrain during development

Homa Adle-Biassette et al.

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2006)

添加到收藏夹

Review Medicine, Research & Experimental

The genetic epidemidogy of neurodegenerative disease

L Bertram et al.

JOURNAL OF CLINICAL INVESTIGATION (2005)

添加到收藏夹

Article Neurosciences

The cellular prion protein (PrPC) prevents apoptotic neuronal cell death and mitochondrial dysfunction induced by serum deprivation

BH Kim et al.

MOLECULAR BRAIN RESEARCH (2004)

添加到收藏夹

Article Biochemistry & Molecular Biology

Absence of the prion protein homologue Doppel causes male sterility

A Behrens et al.

EMBO JOURNAL (2002)

添加到收藏夹

Article Genetics & Heredity

Huntington disease phenocopy is a familial prion disease

RC Moore et al.

AMERICAN JOURNAL OF HUMAN GENETICS (2001)

添加到收藏夹

Article Multidisciplinary Sciences

Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding

GP Saborio et al.

NATURE (2001)

添加到收藏夹

Article Biochemistry & Molecular Biology

Lack of evidence to support the association of the human prion gene with schizophrenia

MT Tsai et al.

MOLECULAR PSYCHIATRY (2001)

添加到收藏夹

© Peeref 2019-2024. All rights reserved.