Publications related to Kuru (disease)

Trans‐seeding of Alzheimer‐related tau protein by a yeast prion

Henning Paul-Julius Stahlberg, Zhiva Skachokova

Abnormal tau protein aggregates constitute a hallmark of Alzheimer's disease. The mechanisms underlying the initiation of tau aggregation in sporadic neurodegeneration remain unclear. Here we investigate whether a non-human prion can seed tau aggregation. ...

2022

Reduced NCOR2 expression accelerates androgen deprivation therapy failure in prostate cancer

Gerard Llimos Aubach

This study addresses the roles of nuclear receptor corepressor 2 (NCOR2) in prostate cancer (PC) progression in response to androgen deprivation therapy (ADT). Reduced NCOR2 expression significantly associates with shorter disease-free survival in patients ...

CELL PRESS2021

In Vivo Longitudinal (1)H MRS Study of Transgenic Mouse Models of Prion Disease in the Hippocampus and Cerebellum at 14.1 T

Rolf Gruetter, Cristina Ramona Cudalbu, Mélanie Craveiro

In vivo (1)H MR spectroscopy allows the non invasive characterization of brain metabolites and it has been used for studying brain metabolic changes in a wide range of neurodegenerative diseases. The prion diseases form a group of fatal neurodegenerative d ...

Springer Verlag2015

Ultrafast photoinduced processes in metal - containing molecular complexes and in proteins

Roberto Monni

The aim of this thesis is to investigate the ultrafast inter¿ and intra¿molecular processes, occurring in both metal¿containing molecular complexes and in proteins, by means of ultrafast Transient Absorption (TA) spectroscopy. The first part of the thesis ...

EPFL2015

The Protein-disulfide Isomerase ERp57 Regulates the Steady-state Levels of the Prion Protein

Maurizio Molinari

Although the accumulation of a misfolded and protease-resistant form of the prion protein (PrP) is a key event in prion pathogenesis, the cellular factors involved in its folding and quality control are poorly understood. PrP is a glycosylated and disulfid ...

Amer Soc Biochemistry Molecular Biology Inc2015

Multicontrast connectometry: A new tool to assess cerebellum alterations in early relapsing‐remitting multiple sclerosis

Jean-Philippe Thiran, Dimitri Nestor Alice Van De Ville, David Paul Roger Romascano, Alessandro Daducci, Cristina Granziera, Djalel Eddine Meskaldji, Jonas Richiardi, Gunnar Krüger, Alexis Roche, Guillaume Bonnier, Ying-Chia Lin, Gloria Menegaz

Background: Cerebellar pathology occurs in late multiple sclerosis (MS) but little is known about cerebellar changes during early disease stages. In this study, we propose a new multicontrast “connectometry” approach to assess the structural and functional ...

Wiley-Blackwell2015

Two Misfolding Routes for the Prion Protein around pH 4.5

Ursula Röthlisberger, Ivano Tavernelli

Using molecular dynamics simulations, we show that the prion protein (PrP) exhibits a dual behavior, with two possible transition routes, upon protonation of H187 around pH 4.5, which mimics specific conditions encountered in endosomes. Our results suggest ...

Public Library Science2013

Prion versus Doppel Protein Misfolding: New Insights from Replica-Exchange Molecular Dynamics Simulations

Ursula Röthlisberger, Ivano Tavernelli

The doppel (Dpl) and prion (PrP) proteins share a very similar fold (three helices and two short beta-strands), while they differ significantly in sequence (only 25% homologous) and in disease-related beta-rich conformations that occur for PrP only. In a p ...

American Chemical Society2013

Enhanced Sampling Molecular Dynamics Identifies PrPSc Structures Harboring a C-Terminal beta-Core

Ursula Röthlisberger, Ivano Tavernelli, Maria Carola Colombo

We perform a replica exchange molecular dynamics simulation corresponding to a 2.8 mu s total time for the extensive enhanced sampling of the conformational space of the C-terminal part (residues 124-226) of the mouse prion protein (PrP); 1.3% of the confo ...

Amer Chemical Soc2012

Characterisation of Living Matter Using Advanced Spectroscopy Techniques

Markus Bonda

In the context of an ageing society neurodegenerative disease have become more and more frequent among humans. Opposing the benefits of a longer life, these diseases have thus triggered research on neurons and how they interact with each other. In vitro as ...

EPFL2011

Nucleation Dependent Polymerization of Amyloid-ß (Aß) as an Important Determinant of Aß Amyloid Formation and Neurotoxicity : Implications for the Pathogenesis of Alzheimer's Disease and Design of Therapies

Mohammad Asad Jan Qureshi

Aggregation and fibril formation of amyloid-β (Aβ) peptides play a pivotal role in the pathogenesis of Alzheimer's disease (AD). Aβ peptides, principally comprising of 40 or 42 amino acid residues (Aβ40 and Aβ42), are produced by proteolytic processing of ...

EPFL2010

Understanding amyloid aggregation by statistical analysis of atomic force microscopy images

Giovanni Dietler, Paolo De Los Rios, Jérôme Flakowski, Raffaele Mezzenga

The aggregation of proteins is central to many aspects of daily life, including food processing, blood coagulation, eye cataract formation disease and prion-related neurodegenerative infections[1–5]. However, the physical mechanisms responsible for amyloid ...

Nature Publishing Group2010

Structure and function of the molecular chaperone Hsp104 from yeast

Hilal Lashuel

The molecular chaperone Hsp104 plays a central role in the clearance of aggregates after heat shock and the propagation of yeast prions. Hsp104's disaggregation activity and prion propagation have been linked to its ability to resolubilize or remodel prote ...

Wiley-Blackwell2009

Immunoisolated cell implants for intracerebral delivery of anti-Aß single chain Fv antibodies in the APP23 Alzheimer's mouse model

Osiris Marroquin Belaunzaran

Alzheimer's disease (AD) is the most prevalent neurodegenerative disease in the elderly population. AD currently affects approximately 27 million people worldwide. A pathological hallmark of AD is the accumulation and deposition of the amyloid beta (Aβ) pe ...

EPFL2009

Solute energy based REMD : developments and applications to prion protein misfold predictions

Molecular dynamics (MD) simulations have increasingly contributed to the understanding of biomolecular processes, allowing for predictions of thermodynamic and structural properties. Unfortunately, the holy grail of protein structure prediction was soon fo ...

EPFL2008