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Prion Strain Mutation and Selection

Science (American Association for the Advancement of Science), 2010-05, Vol.328 (5982), p.1111-1112

Collinge, John

2010

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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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2.7 Å cryo-EM structure of ex vivo RML prion fibrils

Nature communications, 2022-07, Vol.13 (1), p.4004-4004, Article 4004

2022

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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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Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked

Nature communications, 2014-07, Vol.5 (1), p.4347, Article 4347

2014

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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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Two mouse models of Alzheimer’s disease accumulate amyloid at different rates and have distinct Aβ oligomer profiles unaltered by ablation of cellular prion protein

PloS one, 2023-11, Vol.18 (11), p.e0294465-e0294465

2023

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Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

Science (American Association for the Advancement of Science), 2003-10, Vol.302 (5646), p.871-874

2003

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Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease

Proceedings of the National Academy of Sciences - PNAS, 2008-07, Vol.105 (29), p.10238-10243

2008

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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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A blood miRNA signature associates with sporadic Creutzfeldt-Jakob disease diagnosis

Nature communications, 2020-08, Vol.11 (1), p.3960-3960, Article 3960

2020

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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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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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A novel and rapid method for obtaining high titre intact prion strains from mammalian brain

Scientific reports, 2015-05, Vol.5 (1), p.10062-10062, Article 10062

2015

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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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Location and Properties of Metal-Binding Sites on the Human Prion Protein

Proceedings of the National Academy of Sciences - PNAS, 2001-07, Vol.98 (15), p.8531-8535

2001

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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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Prion Strain Mutation and Selection

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

2.7 Å cryo-EM structure of ex vivo RML prion fibrils

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

Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked

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

Two mouse models of Alzheimer’s disease accumulate amyloid at different rates and have distinct Aβ oligomer profiles unaltered by ablation of cellular prion protein

Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease

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

A blood miRNA signature associates with sporadic Creutzfeldt-Jakob disease diagnosis

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

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

A novel and rapid method for obtaining high titre intact prion strains from mammalian brain

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

Location and Properties of Metal-Binding Sites on the Human Prion Protein

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

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