| 1 |
Twenty-year-old African American woman with prion disease associated with the G114V <i>PRNP</i> variant. ( 29577079
)
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Margolesky J....Saporta M.
|
2018 |
| 2 |
Microglia Are Critical in Host Defense Against Prion Disease. ( 29769333
)
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Carroll J.A....Chesebro B.
|
2018 |
| 3 |
Prion disease: Skin is a source of infectious prions in sCJD. ( 29217825
)
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Starling S.
|
2018 |
| 4 |
Autologous neural progenitor cell transplantation into newborn mice modeling for E200K genetic prion disease delays disease progression. ( 29494865
)
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Frid K....Gabizon R.
|
2018 |
| 5 |
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Takada L.T....Geschwind M.D.
|
2018 |
| 6 |
Presumptive BSE cases with an aberrant prion protein phenotype in Switzerland, 2011: Lack of prion disease in experimentally inoculated cattle and bovine prion protein transgenic mice. ( 29675959
)
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Serra F....Seuberlich T.
|
2018 |
| 7 |
Acute Neurotoxicity Models of Prion Disease. ( 29393619
)
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T Islam A.M....Collins S.J.
|
2018 |
| 8 |
Efficient prion disease transmission through common environmental materials. ( 29330304
)
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Pritzkow S....Soto C.
|
2018 |
| 9 |
Prion disease: Dramatic increases in blood levels of tau and neurofilament light in patients with prion disease. ( 29545623
)
|
Ridler C.
|
2018 |
| 10 |
Cerebrospinal Fluid Prion Disease Biomarkers in Pre-clinical and Clinical Naturally Occurring Scrapie. ( 29572672
)
|
|
2018 |
| 11 |
MicroRNA-16 targets mRNA involved in neurite extension and branching in hippocampal neurons during presymptomatic prion disease. ( 29277556
)
|
|
2018 |
| 12 |
Chronic wasting disease: an evolving prion disease of cervids. ( 29887133
)
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|
2018 |
| 13 |
Decrease of RyR2 in the prion infected cell line and in the brains of the scrapie infected mice models and the patients of human prion diseases. ( 29676187
)
|
|
2018 |
| 14 |
Prion Disease in Dromedary Camels, Algeria. ( 29652245
)
|
|
2018 |
| 15 |
Alterations in neuronal metabolism contribute to the pathogenesis of prion disease. ( 29915278
)
|
|
2018 |
| 16 |
The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature. ( 29304167
)
|
Caine D...Mead S
|
2018 |
| 17 |
Treatment with a non-toxic, self-replicating anti-prion delays or prevents prion disease in vivo. ( 28630454
)
|
Diaz-Espinoza R...Soto C
|
2018 |
| 18 |
MicroRNA Alterations in the Brain and Body Fluids of Humans and Animal Prion Disease Models: Current Status and Perspectives. ( 30083102
)
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Kanata E...Llorens F
|
2018 |
| 19 |
Post-mortem magnetic resonance imaging in patients with suspected prion disease: Pathological confirmation, sensitivity, specificity and observer reliability. A national registry. ( 30086144
)
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Gibson LM...Wardlaw JM
|
2018 |
| 20 |
Application of the fragment molecular orbital method to discover novel natural products for prion disease. ( 30166585
)
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Choi J...Sohn HJ
|
2018 |
| 21 |
Muskelin Coordinates PrPC Lysosome versus Exosome Targeting and Impacts Prion Disease Progression. ( 30174115
)
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Heisler FF...Kneussel M
|
2018 |
| 22 |
Structural basis for the complete resistance of the human prion protein mutant G127V to prion disease. ( 30181558
)
|
Zheng Z...Lin D
|
2018 |
| 23 |
The most problematic symptoms of prion disease - an analysis of carer experiences. ( 30353798
)
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Ford L...Mead S
|
2018 |
| 24 |
Cellular and Molecular Mechanisms of Prion Disease. ( 30355150
)
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Sigurdson CJ...Glatzel M
|
2018 |
| 25 |
Mitochondrial dysfunction in preclinical genetic prion disease: A target for preventive treatment? ( 30423473
)
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Keller G...Gabizon R
|
2018 |
| 26 |
Therapeutic strategies for prion disease: a practical perspective. ( 30508662
)
|
Appleby BS...Wang H
|
2018 |
| 27 |
Ganglioside synthase knock-out reduces prion disease incubation time in mouse models. ( 30553837
)
|
Kobayashi A...Miyoshi I
|
2018 |
| 28 |
Stimulations of the Culture Medium of Activated Microglia and TNF-Alpha on a Scrapie-Infected Cell Line Decrease the Cell Viability and Induce Marked Necroptosis That Also Occurs in the Brains from the Patients of Human Prion Diseases. ( 30399321
)
|
Ma Y...Dong XP
|
2018 |
| 29 |
Lysosomal Quality Control in Prion Diseases. ( 28421536
)
|
Majumder P...Chakrabarti O
|
2018 |
| 30 |
Myelin Basic Protein Citrullination, a Hallmark of Central Nervous System Demyelination, Assessed by Novel Monoclonal Antibodies in Prion Diseases. ( 28470584
)
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Jang B...Choi EK
|
2018 |
| 31 |
Toward Therapy of Human Prion Diseases. ( 28961066
)
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Aguzzi A...Frontzek K
|
2018 |
| 32 |
Translational Research in Alzheimer's and Prion Diseases. ( 29172000
)
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Di Fede G...Tagliavini F
|
2018 |
| 33 |
An Amino Acid Substitution Found in Animals with Low Susceptibility to Prion Diseases Confers a Protective Dominant-Negative Effect in Prion-Infected Transgenic Mice. ( 29264770
)
|
Otero A...Castilla J
|
2018 |
| 34 |
Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases. ( 29329906
)
|
Munesue Y...Kobayashi A
|
2018 |
| 35 |
Impaired transport of intrinsically disordered proteins through the Sec61 and SecY translocon; implications for prion diseases. ( 29388511
)
|
Jung S...Tatzelt J
|
2018 |
| 36 |
Cerebrospinal fluid neurofilament light levels in neurodegenerative dementia: Evaluation of diagnostic accuracy in the differential diagnosis of prion diseases. ( 29391125
)
|
Zerr I...Llorens F
|
2018 |
| 37 |
Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein. ( 29458424
)
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Race B...Chesebro B
|
2018 |
| 38 |
THERPA: A small molecule database related to prion protein regulation and prion diseases progression. ( 29633896
)
|
Lee SM...Kim SY
|
2018 |
| 39 |
Glial alterations in human prion diseases: A correlative study of astroglia, reactive microglia, protein deposition, and neuropathological lesions. ( 29642165
)
|
Monz?n M...Badiola JJ
|
2018 |
| 40 |
Effects of peptidyl-prolyl isomerase 1 depletion in animal models of prion diseases. ( 29676205
)
|
Legname G...Moda F
|
2018 |
| 41 |
Regulation of MicroRNAs-Mediated Autophagic Flux: A New Regulatory Avenue for Neurodegenerative Diseases With Focus on Prion Diseases. ( 29867448
)
|
Shah SZA...Yang L
|
2018 |
| 42 |
Differential diagnosis with other rapid progressive dementias in human prion diseases. ( 29887146
)
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Geschwind MD...Murray K
|
2018 |
| 43 |
The zoonotic potential of animal prion diseases. ( 29887151
)
|
Houston F...Andr?oletti O
|
2018 |
| 44 |
Experimental models of human prion diseases and prion strains. ( 29887156
)
|
Diack AB...Bartz JC
|
2018 |
| 45 |
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases. ( 29998397
)
|
Hirsch TZ...Mouillet-Richard S
|
2018 |
| 46 |
Cerebrospinal Fluid Total Prion Protein in the Spectrum of Prion Diseases. ( 30062673
)
|
Villar-Piqu? A...Llorens F
|
2018 |
| 47 |
Lithium as a disease-modifying agent for prion diseases. ( 30135493
)
|
Rela?o-Gin?s A...Crozet C
|
2018 |
| 48 |
Analysis of 22 Years of Surveillance for Prion Diseases in Slovenia, 1996 to 2017. ( 30294364
)
|
?ak? Jager N...Kraigher A
|
2018 |
| 49 |
p62-Keap1-NRF2-ARE Pathway: A Contentious Player for Selective Targeting of Autophagy, Oxidative Stress and Mitochondrial Dysfunction in Prion Diseases. ( 30337853
)
|
Shah SZA...Yang L
|
2018 |
| 50 |
|
Tee BL...Geschwind MD
|
2018 |
