Concept

Protéinopathie

Publications associées (162)

TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates

Henning Paul-Julius Stahlberg, Amanda Jennifer Lewis, Tariq Afroz

Accumulation of abnormally phosphorylated TDP-43 (pTDP-43) is the main pathology in affected neurons of people with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Morphological diversity and neuroanatomical distribution o ...

Springer Nature2018

Cryo-EM structure of alpha-synuclein fibrils

Henning Paul-Julius Stahlberg, Ricardo Cesar Guerrero Ferreira

Parkinson's disease is a progressive neuropathological disorder that belongs to the class of synucleinopathies, in which the protein alpha-synuclein is found at abnormally high concentrations in affected neurons. Its hallmark are intracellular inclusions c ...

eLife Sciences Publications, Ltd2018

Amyloid Single Cell Cytotoxicity Assays by Nanomotion Detection

Giovanni Dietler, Hilal Lashuel, Sandor Kasas, Anne-Laure Mahul Mellier, Giovanni Longo, Francesco Simone Ruggeri

Cells are extremely complex systems able to modify actively their metabolism and behaviour in response to environmental conditions and stimuli, such as pathogenic agents or drugs. The comprehension of these responses is central to understand the molecular ...

Springer Nature2017

Enhancing mitochondrial proteostasis reduces amyloid-beta proteotoxicity

Johan Auwerx, Davide D'Amico, Adrian Schmid, Norman Lionel Moullan, Vincenzo Sorrentino, Hongbo Zhang, Francesca Potenza, Solene Rietsch

Alzheimer's disease is a common and devastating disease characterized by aggregation of the amyloid-beta peptide. However, we know relatively little about the underlying molecular mechanisms or how to treat patients with Alzheimer's disease. Here we provid ...

Nature Publishing Group2017

Polyglutamine expansion affects huntingtin conformation in multiple Huntington’s disease models

Hilal Lashuel, Lara Petricca, Sean Michael Deguire

Conformational changes in disease-associated or mutant proteins represent a key pathological aspect of Huntington's disease (HD) and other protein misfolding diseases. Using immunoassays and biophysical approaches, we and others have recently reported that ...

Springer Nature2017

Administrations of human adult ischemia-tolerant mesenchymal stem cells and factors reduce amyloid beta pathology in a mouse model of Alzheimer's disease

Theo Lasser, Tristan Bolmont, Taoufiq Harach, Fabien Louis Claude Robert Jammes, Nicolas Jean Marc Antoine Duthilleul, Valentin Laurent Herbert Zufferey

The impact of human adult ischemia-tolerant mesenchymal stem cells (hMSCs) and factors (stem cell factors) on cerebral amyloid beta (Aβ) pathology was investigated in a mouse model of Alzheimer's disease (AD). To this end, hMSCs were administered intraveno ...

Elsevier2017

Glycation potentiates α-synuclein-associated neurodegeneration in synucleinopathies

Hilal Lashuel, Magdalena Zweckstetter, Michael Heinrich

α-Synuclein misfolding and aggregation is a hallmark in Parkinson's disease and in several other neurodegenerative diseases known as synucleinopathies. The toxic properties of α-synuclein are conserved from yeast to man, but the precise underpinnings of th ...

Oxford University Press2017

Nanoplasmonic mid-infrared biosensor for in vitro protein secondary structure detection

Giovanni Dietler, Hilal Lashuel, Hatice Altug, Francesco Simone Ruggeri, Dordaneh Etezadi, John Blaine Warner IV

Plasmonic nanoantennas offer new applications in mid-infrared (mid-IR) absorption spectroscopy with ultrasensitive detection of structural signatures of biomolecules, such as proteins, due to their strong resonant near-fields. The amide I fingerprint of a ...

Springer Nature2017

Reduction of Abeta amyloid pathology in APPPS1 transgenic mice in the absence of gut microbiota

Theo Lasser, Tristan Bolmont, Taoufiq Harach, Nicolas Jean Marc Antoine Duthilleul

Alzheimer’s disease is the most common form of dementia in the western world, however there is no cure available for this devastating neurodegenerative disorder. Despite clinical and experimental evidence implicating the intestinal microbiota in a number o ...

2017

Memory performance-related dynamic brain connectivity indicates pathological burden and genetic risk for Alzheimer's disease

Dimitri Nestor Alice Van De Ville, Maria Giulia Preti

Background: The incidence of Alzheimer's disease (AD) strongly relates to advanced age and progressive deposition of cerebral amyloid-beta (A beta), hyperphosphorylated tau, and iron. The purpose of this study was to investigate the relationship between ce ...

Biomed Central Ltd2017

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