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Distinct responses of neurons and astrocytes to TDP-43 proteinopathy in amyotrophic lateral sclerosis

Brain (London, England : 1878), 2020-02, Vol.143 (2), p.430-440

2020

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PrP is a central player in toxicity mediated by soluble aggregates of neurodegeneration-causing proteins

Acta neuropathologica, 2020-03, Vol.139 (3), p.503-526

2020

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Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases

Scientific reports, 2021-03, Vol.11 (1), p.5231-5231, Article 5231

2021

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Prion protein gene mutation detection using long-read Nanopore sequencing

Scientific reports, 2022-05, Vol.12 (1), p.8284-8284, Article 8284

2022

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A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody

Scientific reports, 2022-06, Vol.12 (1), p.9493-12, Article 9493

2022

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Structural features distinguishing infectious ex vivo mammalian prions from non-infectious fibrillar assemblies generated in vitro

Scientific reports, 2019-01, Vol.9 (1), p.376-376, Article 376

2019

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Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

Nature genetics, 2005-08, Vol.37 (8), p.806-808

2005

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The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature

PloS one, 2018-01, Vol.13 (1), p.e0190818-e0190818

2018

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Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy

Scientific reports, 2022-10, Vol.12 (1), p.17198-17198, Article 17198

2022

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Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS

PLoS biology, 2020-06, Vol.18 (6), p.e3000725-e3000725

2020

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Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia

Acta neuropathologica, 2013-09, Vol.126 (3), p.401-409

2013

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Monoclonal antibodies inhibit prion replication and delay the development of prion disease

Nature (London), 2003-03, Vol.422 (6927), p.80-83

2003

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Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis

PloS one, 2010-05, Vol.5 (5), p.e10627

2010

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FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration

Acta neuropathologica, 2010-07, Vol.120 (1), p.33-41

2010

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Case report of homozygous E200D mutation of PRNP in apparently sporadic Creutzfeldt-Jakob disease

BMC neurology, 2021-06, Vol.21 (1), p.248-248, Article 248

2021

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

Brain (London, England : 1878), 2012-02, Vol.135 (2), p.e209-e209

2012

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Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice

Brain (London, England : 1878), 2012-03, Vol.135 (Pt 3), p.819-832

2012

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The Medical Research Council Prion Disease Rating Scale: a new outcome measure for prion disease therapeutic trials developed and validated using systematic observational studies

Brain (London, England : 1878), 2013-04, Vol.136 (Pt 4), p.1116-1127

2013

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Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP

Human molecular genetics, 2012-04, Vol.21 (8), p.1897-1906

2012

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MRI findings are often missed in the diagnosis of Creutzfeldt-Jakob disease

BMC neurology, 2012-12, Vol.12 (1), p.153-153, Article 153

2012

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Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS

PLoS pathogens, 2015-07, Vol.11 (7), p.e1004953-e1004953

2015

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In vitro screen of prion disease susceptibility genes using the scrapie cell assay

Human molecular genetics, 2014-10, Vol.23 (19), p.5102-5108

2014

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Molecular classification of sporadic Creutzfeldt–Jakob disease

Brain (London, England : 1878), 2003-06, Vol.126 (6), p.1333-1346

2003

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Predictive testing for inherited prion disease: report of 22 years experience

European journal of human genetics : EJHG, 2014-12, Vol.22 (12), p.1351-1356

2014

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NT1-Tau Is Increased in CSF and Plasma of CJD Patients, and Correlates with Disease Progression

Cells (Basel, Switzerland), 2021-12, Vol.10 (12), p.3514

2021

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Distinct responses of neurons and astrocytes to TDP-43 proteinopathy in amyotrophic lateral sclerosis

PrP is a central player in toxicity mediated by soluble aggregates of neurodegeneration-causing proteins

Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases

Prion protein gene mutation detection using long-read Nanopore sequencing

A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody

Structural features distinguishing infectious ex vivo mammalian prions from non-infectious fibrillar assemblies generated in vitro

Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature

Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy

Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS

Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia

Monoclonal antibodies inhibit prion replication and delay the development of prion disease

Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis

FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration

Case report of homozygous E200D mutation of PRNP in apparently sporadic Creutzfeldt-Jakob disease

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

Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice

The Medical Research Council Prion Disease Rating Scale: a new outcome measure for prion disease therapeutic trials developed and validated using systematic observational studies

Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP

MRI findings are often missed in the diagnosis of Creutzfeldt-Jakob disease

Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS

In vitro screen of prion disease susceptibility genes using the scrapie cell assay

Molecular classification of sporadic Creutzfeldt–Jakob disease

Predictive testing for inherited prion disease: report of 22 years experience

NT1-Tau Is Increased in CSF and Plasma of CJD Patients, and Correlates with Disease Progression

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