A5028003116- Amyloidosis: Diagnosis, Treatment, Outcomes
- Music Technology and Sound Studies
- Alzheimer's disease research and treatments
- Modeling, Simulation, and Optimization
- Mechanics and Biomechanics Studies
- Protein Kinase Regulation and GTPase Signaling
- Music and Audio Processing
- Advanced Malware Detection Techniques
- Cardiac electrophysiology and arrhythmias
- Protein Structure and Dynamics
- Tactile and Sensory Interactions
- 14-3-3 protein interactions
Université de Limoges
2025
Centre National de la Recherche Scientifique
2025
Contrôle de la Réponse Immune B et Lymphoproliférations
2024-2025
Amyloidosis Foundation
2025
Inserm
2025
University of Music and Performing Arts Vienna
2023-2024
University of Pavia
2024
AL amyloidosis is a life-threatening disease caused by deposition of immunoglobulin light chains. Whilst the mechanisms underlying chains amyloidogenesis in vivo remain unclear, several studies have highlighted role that tissue environment and structural amyloidogenicity individual pathogenesis.AL natural deposits contain both full-length fragments encompassing variable domain (VL) as well different length segments constant region (CL), thus highlighting relevance proteolysis may...
Abstract Immunoglobulin light chain (LC) amyloidosis (AL) is one of the most common types systemic but there no reliable in vivo model for better understanding this disease. Here, we develop a transgenic mouse producing human AL LC. We show that soluble full length LC not toxic single injection pre-formed amyloid fibrils or an unstable fragment leads to deposits associated with early cardiac dysfunction. mice are highly similar human, arguing conserved mechanism formation. Overall, closely...
ABSTRACT AL amyloidosis is one of the most common types systemic amyloidosis, caused by deposition in tissues fibrillar aggregates abnormal immunoglobulin (Ig) light chain (LC), leading to organ dysfunction. The frequent and severe forms affect kidneys heart, latter being associated with a poor prognosis. Despite extensive efforts decipher mechanisms fibril formation their toxicity, lack reliable vivo models hinders study disease its physiological context. We developped transgenic mouse...
In bowed-string instruments, the force exerted by string on bridge may be used for analyzing excitation process. This is caused vibration of due to musician's bowing action. The dynamic component this has been investigated in relevant literature. study, focusing a monochord, an approach proposed simultaneously measure both static and components rigid support. use robotic arm excite via linear action permits under controlled conditions. are analyzed transverse plane at several moments bow...
The playability limits in the bow force and acceleration parameter space (usually visualized as a Guettler diagram) define conditions for establishing Helmholtz motion during bowed attacks bowed-string instruments. Despite few theoretical numerical studies, there is little empirical validation of these limits. experimental scanning diagram tedious process, it requires use bowing machine to control parameters. This study proposes method collecting analyzing data produce measured diagrams....