Benoit I. Giasson

Corpora amylacea profiling reveals disease stage and brain region-specific alterations in glycogen metabolism in Alzheimer’s disease patient brains

Jonathan Anthony B. Villareal, Tim Bathe, Ana de la Rosa, Rohan V. Sharma et autres

Increasing evidence indicates that shifts in brain polysaccharide metabolism can influence the progression of multiple neurodegenerative diseases. Here, we profile corpora amylacea (CA) in 230 postmortem human brains, revealing significantly higher densities in Alzheimer's disease (AD) patients when compared with patients with …

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Correction to: Induction of CNS α-synuclein pathology by fibrillar and non-amyloidogenic recombinant α-synuclein

Amanda N. Sacino, Mieu Brooks, Nicholas H. McGarvey, Alex B. McKinney et autres

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Rigorous evaluation of pathological α-synucleinopathy transmissibility between animals in an experimental model

Amanda N. Sacino, Todd Eliot Golde, David Borchelt, Benoit I. Giasson et autres

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Progressive Supranuclear Palsy PERK Haplotype B Selectively Translates DLX1 Promoting Tau Toxicity

Christian B. Lessard, Diego Rubio Rubio, Samantha G Tolton, Marangelie Criado-Marrero et autres

The unfolded protein response (UPR) sensor PERK exists in haplotypes A and B. PERK-B confers increased risk for tauopathies like progressive supranuclear palsy (PSP), but the mechanisms distinguishing its function from PERK-A and contributing to its association with tauopathy remain unknown. Here, …

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C1q-dependent clearance of alpha-synuclein allows macrophages to transiently limit enteric synucleinopathy in male mice

Phillip M. Mackie, Joanne M. Koshy, M Bhogade, T Hammoor et autres

Deposition of misfolded α-synuclein (αsyn) in the enteric nervous system (ENS) is found in multiple neurodegenerative diseases. It is hypothesized that ENS synucleinopathy contributes to both the pathogenesis and non-motor morbidity in Parkinson’s Disease (PD), but the cellular and molecular mechanisms that …

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Identification and Functional Characterization of the Axolotl ( Ambystoma mexicanum ) Tau Protein

Zachary Strickland, Benoit I. Giasson, Jada Lewis

BACKGROUND: Tauopathies are characterized by abnormal aggregation of tau that results in neuronal dysfunction and degeneration. Developing strategies to preserve existing neuronal function, prevent neurodegeneration, or restore lost neurons may have the most potential to combat human tauopathy; however, the adult human …

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Parkinson’s paradox: alpha-synuclein’s selective strike on SNc dopamine neurons over VTA

Liem Minh Phan, Douglas R. Miller, Adithya Gopinath, Min Lin et autres

A central question in Parkinson's disease (PD) and related synucleinopathies research is why dopamine neurons in the substantia nigra pars compacta (SNc) are more vulnerable than those in the ventral tegmental area (VTA). We investigated how α-synuclein affects neuronal activity before cell …

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Progressive Supranuclear Palsy PERK haplotype B selectively translates DLX1 promoting tau toxicity

Christian B. Lessard, Diego Rubio-Rubio, Samantha G Tolton, Marangelie Criado‐Marrero et autres

The unfolded protein response sensor PERK exists in haplotypes A and B. PERK-B confers increased risk for tauopathies like progressive supranuclear palsy (PSP), but the mechanisms distinguishing its function from PERK-A and contributing to its association with tauopathy remain unknown. Here, we …

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A novel pThr217 tau monoclonal antibody reveals neuropathological heterogeneity in tauopathies

Giavanna Paterno, Brach M. Bell, Kimberly-Marie M. Gorion, Stephan S. Quintin et autres

Tauopathies are a group of clinically and neuropathologically diverse neurodegenerative disorders defined by the abnormal aggregation of tau protein. While tau is normally soluble with limited secondary structure, pathological tau is characterized by hyperphosphorylation and assembly into fibrils which accumulate in neurons …

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Tau from SPAM Transgenic Mice Exhibit Potent Strain-Specific Prion-Like Seeding Properties Characteristic of Human Neurodegenerative Diseases

Ethan D. Smith, Giavanna Paterno, Brach M. Bell, Kimberly-Marie M. Gorion et autres

Tauopathies, including Alzheimer's disease and frontotemporal dementia with Parkinsonism linked to chromosome 17 (FTDP-17), are characterized by the aberrant aggregation of tau protein into neurofibrillary tangles. Despite extensive studies on tau aggregation, the mechanisms of tau misfolding and propagation remain incompletely understood. …

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Uncovering α-synuclein and tau burden in Alzheimer’s and Lewy body diseases

Stephan S. Quintin, Giavanna Paterno, Grace M. Lloyd, Stefan Prokop et autres

Neurodegenerative diseases are classified based on their histopathological hallmarks alongside their clinical manifestations. Alzheimer's disease and Lewy body disease, which includes Parkinson's disease, are traditionally referred to as tauopathy and synucleinopathy, respectively. However, much like how Alzheimer's disease can present with Parkinsonian …

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Neuronal tissue collection from intra-cranial instruments used in deep brain stimulation surgery for Parkinson’s disease with implications for study of alpha-synuclein

Zachary A. Sorrentino, Joshua Riklan, Grace M. Lloyd, Brandon P. Lucke-Wold et autres

Alpha-synuclein (αSyn) forms pathologic aggregates in Parkinson's disease (PD) and is implicated in mechanisms underlying neurodegeneration. While pathologic αSyn has been extensively studied, there is currently no method to evaluate αSyn within the brains of living patients. Patients with PD are often …

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