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Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease.

Peter Hermann, Brian Appleby, Jean-Philippe Brandel, Byron Caughey, Steven Collins, Michael D Geschwind, Alison Green, Stephane Haïk, Gabor G Kovacs, Anna Ladogana, Franc Llorens, Simon Mead, Noriyuki Nishida, Suvankar Pal, Piero Parchi, Maurizio Pocchiari, Katsuya Satoh, Gianluigi Zanusso, Inga Zerr,

Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrP<sup>Sc</sup>). Effective therapeutics are currently not available and accurate diagnosis can be challenging. Clinical diagnostic criteria use a combination of characteristic neuropsychiatric symptoms, CSF proteins 14-3-3, MRI, and EEG. Supportive biomarkers, such as high CSF total tau, ... Read more >>

Lancet Neurol (The Lancet. Neurology)
[2021, 20(3):235-246]

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Brazilin Removes Toxic alpha-Synuclein and Seeding Competent Assemblies from Parkinson Brain by Altering Conformational Equilibrium.

George R Nahass, Yuanzi Sun, Yong Xu, Mark Batchelor, Madeleine Reilly, Iryna Benilova, Niraja Kedia, Kevin Spehar, Frank Sobott, Richard B Sessions, Byron W Caughey, Sheena E Radford, Parmjit Jat, John Collinge, Jan Bieschke,

Alpha-synuclein (α-syn) fibrils, a major constituent of the neurotoxic Lewy Bodies in Parkinson's disease, form via nucleation dependent polymerization and can replicate by a seeding mechanism. Brazilin, a small molecule derived from red cedarwood trees in Brazil, has been shown to inhibit the fibrillogenesis of amyloid-beta (Aβ) and α-syn as ... Read more >>

J Mol Biol (Journal of molecular biology)
[2021, :166878]

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Structure of an infectious mammalian prion

Allison Kraus, Forrest Hoyt, Cindi Schwartz, Bryan Hansen, Andrew Hughson, Efrosini Artikis, Brent Race, Byron Caughey,

<h4>ABSTRACT</h4> Classical mammalian prions are assemblies of prion protein molecules that are extraordinarily transmissible, with a microgram of protein containing up to 10 8 lethal doses of infectivity 1,2 . Unlike most other pathogenic and amyloidogenic proteins, prions typically contain glycolipid anchors 3 and abundant asparagine-linked glycans 4-6 . The ... Read more >>

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Correction to: Rapid and ultra-sensitive quantitation of disease-associated α-synuclein seeds in brain and cerebrospinal fluid by αSyn RT-QuIC.

Bradley R Groveman, Christina D Orrù, Andrew G Hughson, Lynne D Raymond, Gianluigi Zanusso, Bernardino Ghetti, Katrina J Campbell, Jiri Safar, Douglas Galasko, Byron Caughey,

An amendment to this paper has been published and can be accessed via the original article. ... Read more >>

Acta Neuropathol Commun (Acta neuropathologica communications)
[2020, 8(1):180]

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Accommodation of In-Register N-Linked Glycans on Prion Protein Amyloid Cores.

Efrosini Artikis, Amitava Roy, Hugo Verli, Yraima Cordeiro, Byron Caughey,

Although prion protein fibrils can have either parallel-in-register intermolecular β-sheet (PIRIBS) or, probably, β-solenoid architectures, the plausibility of PIRIBS architectures for the usually glycosylated natural prion strains has been questioned based the expectation that such glycans would not fit if stacked in-register on each monomer within a fibril. To directly ... Read more >>

ACS Chem Neurosci (ACS chemical neuroscience)
[2020, 11(24):4092-4097]

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Skin α-Synuclein Aggregation Seeding Activity as a Novel Biomarker for Parkinson Disease.

Zerui Wang, Katelyn Becker, Vincenzo Donadio, Sandra Siedlak, Jue Yuan, Masih Rezaee, Alex Incensi, Anastasia Kuzkina, Christina D Orrú, Curtis Tatsuoka, Rocco Liguori, Steven A Gunzler, Byron Caughey, Maria E Jimenez-Capdeville, Xiongwei Zhu, Kathrin Doppler, Li Cui, Shu G Chen, Jiyan Ma, Wen-Quan Zou,

<h4>Importance</h4>Deposition of the pathological α-synuclein (αSynP) in the brain is the hallmark of synucleinopathies, including Parkinson disease (PD), Lewy body dementia (LBD), and multiple system atrophy (MSA). Whether real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA) assays can sensitively detect skin biomarkers for PD and non-PD synucleinopathies remains ... Read more >>

JAMA Neurol (JAMA neurology)
[2020, :]

Cited: 2 times

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Risk of Transmissibility From Neurodegenerative Disease-Associated Proteins: Experimental Knowns and Unknowns.

David M Asher, Ermias Belay, Eileen Bigio, Sebastian Brandner, Scott A Brubaker, Byron Caughey, Brychan Clark, Inger Damon, Marc Diamond, Michelle Freund, Bradley T Hyman, Mathias Jucker, C Dirk Keene, Andrew P Lieberman, Miroslaw Mackiewicz, Thomas J Montine, Susan Morgello, Creighton Phelps, Jiri Safar, Julie A Schneider, Lawrence B Schonberger, Christina Sigurdson, Nina Silverberg, John Q Trojanowski, Matthew P Frosch,

Recent studies in animal models demonstrate that certain misfolded proteins associated with neurodegenerative diseases can support templated misfolding of cognate native proteins, to propagate across neural systems, and to therefore have some of the properties of classical prion diseases like Creutzfeldt-Jakob disease. The National Institute of Aging convened a meeting ... Read more >>

J Neuropathol Exp Neurol (Journal of neuropathology and experimental neurology)
[2020, 79(11):1141-1146]

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Ring trial of 2nd generation RT-QuIC diagnostic tests for sporadic CJD.

Christina D Orrú, Bradley R Groveman, Aaron Foutz, Matilde Bongianni, Franco Cardone, Neil McKenzie, Audrey Culeux, Anna Poleggi, Katarina Grznarova, Daniela Perra, Michele Fiorini, Xiaoqin Liu, Anna Ladogana, Marco Sbriccoli, Andrew G Hughson, Stéphane Haïk, Alison J Green, Michael D Geschwind, Maurizio Pocchiari, Jiri G Safar, Gianluigi Zanusso, Byron Caughey,

<h4>Objective</h4>Real-time quaking-induced conversion (RT-QuIC) assays detect prion-seeding activity in a variety of human biospecimens, including cerebrospinal fluid and olfactory mucosa swabs. The assay has shown high diagnostic accuracy in patients with prion disorders. Recently, advances in these tests have led to markedly improved diagnostic sensitivity and reduced assay times. Accordingly, ... Read more >>

Ann Clin Transl Neurol (Annals of clinical and translational neurology)
[2020, 7(11):2262-2271]

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Brazilin Removes Toxic alpha-Synuclein and Seeding Competent Assemblies from Parkinson Brain by Altering Conformational Equilibrium

George Nahass, Yuanzi Sun, Yong Xu, Mark Batchelor, Madeleine Reilly, Iryna Benilova, Niraja Kedia, Kevin Spehar, Frank Sobott, Richard Sessions, Byron Caughey, Sheena Radford, Parmjit Jat, John Collinge, Jan Bieschke,

<h4>ABSTRACT</h4> Alpha-synuclein (α-syn) fibrils, a major constituent of the neurotoxic Lewy Bodies in Parkinson’s disease, form via nucleation dependent polymerization and can replicate by a seeding mechanism. Brazilin, a small molecule derived from red cedarwood trees in Brazil, has been shown to inhibit the fibrillogenesis of amyloid-beta (Aβ) and α-syn, ... Read more >>

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Correction to: Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies.

Marcello Rossi, Niccolò Candelise, Simone Baiardi, Sabina Capellari, Giulia Giannini, Christina D Orrù, Elena Antelmi, Angela Mammana, Andrew G Hughson, Giovanna Calandra-Buonaura, Anna Ladogana, Giuseppe Plazzi, Pietro Cortelli, Byron Caughey, Piero Parchi,

he article Ultrasensitive RT‑QuIC assay with high sensitivity and specificity for Lewy body‑. ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2020, 140(2):245]

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Proteopathic Seed Amplification Assays for Neurodegenerative Disorders.

Natália do Carmo Ferreira, Byron Caughey,

The need for etiological biomarkers for neurodegenerative diseases involving protein aggregation has prompted development of ultrasensitive cellular and cell-free assays based on the prion-like seeding capacity of such aggregates. Among them, prion RT-QuIC assays allow accurate antemortem Creutzfeldt-Jakob disease diagnosis using cerebrospinal fluid and nasal brushings. Analogous assays for synucleinopathies ... Read more >>

Clin Lab Med (Clinics in laboratory medicine)
[2020, 40(3):257-270]

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

Gregory J Raymond, Brent Race, Christina D Orrú, Lynne D Raymond, Matilde Bongianni, Michele Fiorini, Bradley R Groveman, Sergio Ferrari, Luca Sacchetto, Andrew G Hughson, Salvatore Monaco, Maurizio Pocchiari, Gianluigi Zanusso, Byron Caughey,

<h4>Objective</h4>The detection of prion seeding activity in CSF and olfactory mucosal brushings using real-time quaking-induced conversion assays allows highly accurate clinical diagnosis of sporadic Creutzfeldt-Jakob disease. To gauge transmission risks associated with these biospecimens and their testing, we have bioassayed prion infectivity levels in patients' brain tissue, nasal brushings, and ... Read more >>

Ann Clin Transl Neurol (Annals of clinical and translational neurology)
[2020, 7(6):932-944]

Cited: 1 time

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Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies.

Marcello Rossi, Niccolò Candelise, Simone Baiardi, Sabina Capellari, Giulia Giannini, Christina D Orrù, Elena Antelmi, Angela Mammana, Andrew G Hughson, Giovanna Calandra-Buonaura, Anna Ladogana, Giuseppe Plazzi, Pietro Cortelli, Byron Caughey, Piero Parchi,

The clinical diagnosis of synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), is challenging, especially at an early disease stage, due to the heterogeneous and often non-specific clinical manifestations. The discovery of reliable specific markers for synucleinopathies would consequently be of great aid ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2020, 140(1):49-62]

Cited: 2 times

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

Angela Mammana, Simone Baiardi, Marcello Rossi, Alessia Franceschini, Vincenzo Donadio, Sabina Capellari, Byron Caughey, Piero Parchi,

Prion real-time quaking-induced conversion (RT-QuIC) is an ultrasensitive assay detecting pathological aggregates of misfolded prion protein in biospecimens. We studied 71 punch biopsy skin samples of 35 patients with Creutzfeldt-Jakob disease (CJD), including five assessed in vitam. The results confirmed the high value of skin prion RT-QuIC for CJD diagnosis ... Read more >>

Ann Clin Transl Neurol (Annals of clinical and translational neurology)
[2020, 7(4):559-564]

Cited: 4 times

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Correction to: 4-Repeat tau seeds and templating subtypes as brain and CSF biomarkers of frontotemporal lobar degeneration.

Eri Saijo, Michael A Metrick, Shunsuke Koga, Piero Parchi, Irene Litvan, Salvatore Spina, Adam Boxer, Julio C Rojas, Douglas Galasko, Allison Kraus, Marcello Rossi, Kathy Newell, Gianluigi Zanusso, Lea T Grinberg, William W Seeley, Bernardino Ghetti, Dennis W Dickson, Byron Caughey,

The original version of this article unfortunately contained a mistake. The Panel A in the published figure 5 is incorrect. The corrected Figure 5 is placed in the following page. ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2020, 139(1):79-81]

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A single ultrasensitive assay for detection and discrimination of tau aggregates of Alzheimer and Pick diseases.

Michael A Metrick, Natália do Carmo Ferreira, Eri Saijo, Allison Kraus, Kathy Newell, Gianluigi Zanusso, Michele Vendruscolo, Bernardino Ghetti, Byron Caughey,

Multiple neurodegenerative diseases are characterized by aggregation of tau molecules. Adult humans express six isoforms of tau that contain either 3 or 4 microtubule binding repeats (3R or 4R tau). Different diseases involve preferential aggregation of 3R (e.g Pick disease), 4R (e.g. progressive supranuclear palsy), or both 3R and 4R ... Read more >>

Acta Neuropathol Commun (Acta neuropathologica communications)
[2020, 8(1):22]

Cited: 6 times

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Transmissibility versus Pathogenicity of Self-Propagating Protein Aggregates.

Byron Caughey, Allison Kraus,

The prion-like spreading and accumulation of specific protein aggregates appear to be central to the pathogenesis of many human diseases, including Alzheimer's and Parkinson's. Accumulating evidence indicates that inoculation of tissue extracts from diseased individuals into suitable experimental animals can in many cases induce the aggregation of the disease-associated protein, ... Read more >>

Viruses (Viruses)
[2019, 11(11):]

Cited: 3 times

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Million-fold sensitivity enhancement in proteopathic seed amplification assays for biospecimens by Hofmeister ion comparisons.

Michael A Metrick, Natalia do Carmo Ferreira, Eri Saijo, Andrew G Hughson, Allison Kraus, Christina Orrú, Michael W Miller, Gianluigi Zanusso, Bernardino Ghetti, Michele Vendruscolo, Byron Caughey,

Recent work with prion diseases and synucleinopathies indicates that accurate diagnostic methods for protein-folding diseases can be based on the ultrasensitive, amplified measurement of pathological aggregates in biospecimens. A better understanding of the physicochemical factors that control the seeded polymerization of such aggregates, and their amplification in vitro, should allow ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2019, 116(46):23029-23039]

Cited: 8 times

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4-Repeat tau seeds and templating subtypes as brain and CSF biomarkers of frontotemporal lobar degeneration.

Eri Saijo, Michael A Metrick, Shunsuke Koga, Piero Parchi, Irene Litvan, Salvatore Spina, Adam Boxer, Julio C Rojas, Douglas Galasko, Allison Kraus, Marcello Rossi, Kathy Newell, Gianluigi Zanusso, Lea T Grinberg, William W Seeley, Bernardino Ghetti, Dennis W Dickson, Byron Caughey,

To address the need for more meaningful biomarkers of tauopathies, we have developed an ultrasensitive tau seed amplification assay (4R RT-QuIC) for the 4-repeat (4R) tau aggregates of progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and other diseases with 4R tauopathy. The assay detected seeds in 10<sup>6</sup>-10<sup>9</sup>-fold dilutions of 4R ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2020, 139(1):63-77]

Cited: 11 times

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Antisense oligonucleotides extend survival of prion-infected mice.

Gregory J Raymond, Hien Tran Zhao, Brent Race, Lynne D Raymond, Katie Williams, Eric E Swayze, Samantha Graffam, Jason Le, Tyler Caron, Jacquelyn Stathopoulos, Rhonda O'Keefe, Lori L Lubke, Andrew G Reidenbach, Allison Kraus, Stuart L Schreiber, Curt Mazur, Deborah E Cabin, Jeffrey B Carroll, Eric Vallabh Minikel, Holly Kordasiewicz, Byron Caughey, Sonia M Vallabh,

Prion disease is a fatal, incurable neurodegenerative disease of humans and other mammals caused by conversion of cellular prion protein (PrP; PrPC) into a self-propagating neurotoxic conformer (prions; PrPSc). Strong genetic proofs of concept support lowering PrP expression as a therapeutic strategy. Antisense oligonucleotides (ASOs) can provide a practical route ... Read more >>

JCI Insight (JCI insight)
[2019, 5:]

Cited: 7 times

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Association of Blood and Cerebrospinal Fluid Tau Level and Other Biomarkers With Survival Time in Sporadic Creutzfeldt-Jakob Disease.

Adam M Staffaroni, Abigail O Kramer, Megan Casey, Huicong Kang, Julio C Rojas, Christina D Orrú, Byron Caughey, I Elaine Allen, Joel H Kramer, Howard J Rosen, Kaj Blennow, Henrik Zetterberg, Michael D Geschwind,

<h4>Importance</h4>Fluid biomarkers that can predict survival time in sporadic Creutzfeldt-Jakob disease (sCJD) will be critical for clinical care and for treatment trials.<h4>Objective</h4>To assess whether plasma and cerebrospinal fluid (CSF) biomarkers are associated with survival time in patients with sCJD.<h4>Design, setting, and participants</h4>In this longitudinal cohort study, data from 193 patients ... Read more >>

JAMA Neurol (JAMA neurology)
[2019, :]

Cited: 10 times

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Early and Pre-Clinical Detection of Prion Seeding Activity in Cerebrospinal Fluid of Goats using Real-Time Quaking-Induced Conversion Assay.

Alessandra Favole, Maria Mazza, Elena Vallino Costassa, Antonio D'Angelo, Guerino Lombardi, Paola Marconi, Paola Crociara, Elena Berrone, Marina Gallo, Claudia Palmitessa, Christina D Orrù, Byron Caughey, Pier L Acutis, Maria Caramelli, Cristina Casalone, Cristiano Corona,

Since 2005, two cases of natural bovine spongiform encephalopathies (BSE) have been reported in goats. Furthermore, experimental transmissions of classical (C-BSE) and atypical (L-BSE) forms of BSE in goats were also reported. To minimize further spreading of prion diseases in small ruminants the development of a highly sensitive and specific ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):6173]

Cited: 2 times

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Ultrasensitive RT-QuIC Seed Amplification Assays for Disease-Associated Tau, α-Synuclein, and Prion Aggregates.

Eri Saijo, Bradley R Groveman, Allison Kraus, Michael Metrick, Christina D Orrù, Andrew G Hughson, Byron Caughey,

The abnormal assembly of tau, α-synuclein (αSyn), or prion protein into oligomers and multimers underpins the molecular pathogenesis of multiple neurodegenerative diseases. Such pathological aggregates can often grow by seeded polymerization mechanisms. We and others have taken advantage of these mechanisms to amplify seeding activities in vitro and devise ultrasensitive, ... Read more >>

Methods Mol Biol (Methods in molecular biology (Clifton, N.J.))
[2019, 1873:19-37]

Cited: 6 times

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Seeding selectivity and ultrasensitive detection of tau aggregate conformers of Alzheimer disease.

Allison Kraus, Eri Saijo, Michael A Metrick, Kathy Newell, Christina J Sigurdson, Gianluigi Zanusso, Bernardino Ghetti, Byron Caughey,

Alzheimer disease (AD) and chronic traumatic encephalopathy (CTE) involve the abnormal accumulation in the brain of filaments composed of both three-repeat (3R) and four-repeat (4R) (3R/4R) tau isoforms. To probe the molecular basis for AD's tau filament propagation and to improve detection of tau aggregates as potential biomarkers, we have ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2019, 137(4):585-598]

Cited: 19 times

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Early preclinical detection of prions in the skin of prion-infected animals.

Zerui Wang, Matteo Manca, Aaron Foutz, Manuel V Camacho, Gregory J Raymond, Brent Race, Christina D Orru, Jue Yuan, Pingping Shen, Baiya Li, Yue Lang, Johnny Dang, Alise Adornato, Katie Williams, Nicholas R Maurer, Pierluigi Gambetti, Bin Xu, Witold Surewicz, Robert B Petersen, Xiaoping Dong, Brian S Appleby, Byron Caughey, Li Cui, Qingzhong Kong, Wen-Quan Zou,

A definitive pre-mortem diagnosis of prion disease depends on brain biopsy for prion detection currently and no validated alternative preclinical diagnostic tests have been reported to date. To determine the feasibility of using skin for preclinical diagnosis, here we report ultrasensitive serial protein misfolding cyclic amplification (sPMCA) and real-time quaking-induced ... Read more >>

Nat Commun (Nature communications)
[2019, 10(1):247]

Cited: 10 times

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