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Concept

Small fiber peripheral neuropathy

Publications associées (20)

Early-life vitamin A treatment rescues neonatal infection-induced durably impaired tolerogenic properties of celiac lymph nodes

Jörn Pezoldt

Gut -draining mesenteric and celiac lymph nodes (mLNs and celLNs) critically contribute to peripheral tolerance toward food and microbial antigens by supporting the de novo induction of regulatory T cells (Tregs). These tolerogenic properties of mLNs and c ...

Cell Press2024

Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy

Bernard Schneider

Spinal muscular atrophy (SMA) is a neuromuscular disease caused by loss of the SMN1 gene and low SMN protein levels. Although lower motor neurons are a primary target, there is evidence that peripheral organ defects contribute to SMA. Current SMA gene ther ...

2022

The integrated stress response contributes to tRNA synthetase-associated peripheral neuropathy

Yu Bai, René Uwe Schneider, Cédric Gobet

Dominant mutations in ubiquitously expressed transfer RNA (tRNA) synthetase genes cause axonal peripheral neuropathy, accounting for at least six forms of Charcot-Marie-Tooth (CMT) disease. Genetic evidence in mouse and Drosophila models suggests a gain-of ...

American Association for the Advancement of Science2021

Gene replacement therapy provides benefit in an adult mouse model of Leigh syndrome

Johan Auwerx, Giorgia Benegiamo

Mutations in nuclear-encoded mitochondrial genes are responsible for a broad spectrum of disorders among which Leigh syndrome is the most common in infancy. No effective therapies are available for this severe disease mainly because of the limited capabili ...

2020

Decoding of grasping tasks from intraneural recordings in trans-radial amputee

Silvestro Micera, Stanisa Raspopovic, Ivo Strauss, Giacomo Valle, Francesco Maria Petrini

Objective. A major challenge in neuroprosthetics is the restoration of sensory-motor hand functions in upper-limb amputees. Neuroprostheses based on the direct re-connection of the peripheral nerves may be an interesting approach for re-establishing the na ...

2020

Schwann cells ER-associated degradation contributes to myelin maintenance in adult nerves and limits demyelination in CMT1B mice

Maurizio Molinari

In the peripheral nervous system (PNS) myelinating Schwann cells synthesize large amounts of myelin protein zero (P0) glycoprotein, an abundant component of peripheral nerve myelin. In humans, mutations in P0 cause the demyelinating Charcot-Marie-Tooth 1B ...

2019

A Adaptive Algorithm for Online Interference Cancellation in EMG Sensors

One of the biggest issues encountered in the analysis of sensitive electromyography (EMG) sensor data is the power line interference (PLI). Conventional methods in literature either lose valuable sensor data or inadequately decrease the power line noise. I ...

2019

Altered interplay between endoplasmic reticulum and mitochondria in Charcot-Marie-Tooth type 2A neuropathy

Bernard Schneider, Mary-Claude Croisier-Coeytaux, Catherine Maclachlan, Roman Chrast, Cylia Cloé Rochat, Nathalie Marie Géraldine Bernard

Mutations in the MFN2 gene encoding Mitofusin 2 lead to the development of Charcot-Marie-Tooth type 2A (CMT2A), a dominant axonal form of peripheral neuropathy. Mitofusin 2 is localized at both the outer membrane of mitochondria and the endoplasmic reticul ...

National Academy of Sciences2019

Microchannel-based technologies for nerve interfacing

Sandra Gribi

Biomedical technologies in neuroprosthetics are made to restore or replace functions from the nervous system, as well as alleviate pathological conditions. Peripheral nerve interfaces use neuromodulatory techniques to inhibit pain or depression, prevent ep ...

EPFL2019

A microfabricated nerve-on-a-chip platform for rapid assessment of neural conduction in explanted peripheral nerve fibers

Stéphanie Lacour, Diego Ghezzi, Sandra Gribi

Peripheral nerves are anisotropic and heterogeneous neural tissues. Their complex physiology restricts realistic in vitro models, and high resolution and selective probing of axonal activity. Here, we present a nerve-on-a-chip platform that enables rapid e ...

Nature Research2018

Optical cuff for optogenetic control of the peripheral nervous system

Stéphanie Lacour, Grégoire Courtine, Léonie Asboth, Frédéric Pierre Gérald Michoud

Objective. Nerves in the peripheral nervous system (PNS) contain axons with specific motor, somatosensory and autonomic functions. Optogenetics offers an efficient approach to selectively activate axons within the nerve. However, the heterogeneous nature o ...

2018

Hybrid peripheral-spinal neuroprosthesis for refined motor execution after paralysis

Sophie Marie Marthe Wurth

Spinal cord injury (SCI) disrupts the communication between the brain and the spinal circuits responsible for movement, thereby causing severe motor deficits. Current strategies to restore function to paralyzed limbs have separately investigated electrical ...

EPFL2018

Long-term usability and bio-integration of polyimide-based intraneural stimulating electrodes

Silvestro Micera, Grégoire Courtine, Marco Capogrosso, Stanisa Raspopovic, Alessandra Piersigilli, Quentin Barraud, Nawal Noëlle Kinany, Jacopo Rigosa, Sophie Marie Marthe Wurth, Jérôme Gandar, Natalia Pavlova

Stimulation of peripheral nerves has transiently restored lost sensation and has the potential to alleviate motor deficits. However, incomplete characterization of the long-term usability and bio-integration of intra-neural implants has restricted their us ...

Elsevier Sci Ltd2017

Genome-wide association study of peripheral neuropathy with D-drug-containing regimens in AIDS Clinical Trials Group protocol 384

Paul Jack Mc Laren, Jun Li

Stavudine (d4T) was, until recently, one of the most widely prescribed antiretroviral drugs worldwide. While there has been a major shift away from d4T use in resource-limited countries, a large number of patients have previously received (or continue to r ...

Springer2014

Steady-State Antigen Scavenging, Cross-Presentation, and CD8(+) T Cell Priming: A New Role for Lymphatic Endothelial Cells

Melody Swartz, Sachiko Hirosue, Marcela Rincón-Restrepo, Patricia Corthésy Henrioud, Efthymia Vokali

Until recently, the known roles of lymphatic endothelial cells (LECs) in immune modulation were limited to directing immune cell trafficking and passively transporting peripheral Ags to lymph nodes. Recent studies demonstrated that LECs can directly suppre ...

American Association of Immunologists ; Oxford University Press2014

Investigating Ocular Movements and Vestibular Evoked Potentials for a Vestibular Neuroprosthesis: Response to Pulse Trains and Baseline Stimulation

Silvestro Micera

No adequate treatment currently exists for bilateral vestibulopathy, which can result in significant decreases of social and physical functioning. To improve patients' quality of life, vestibular neuroprostheses are being developed. Efficacy of current pro ...

Ieee2013

Preserving axosomatic spiking features despite diverse dendritic morphology

Henry Markram, Felix Schürmann, Idan Segev

Throughout the nervous system, cells belonging to a certain electrical class (e-class)-sharing high similarity in firing response properties-may nevertheless have widely variable dendritic morphologies. To quantify the effect of this morphological variabil ...

American Physiological Society2013

Magnetic resonance imaging of peripheral nerves: Differences in magnetization transfer

Gunnar Krüger, Giulio Gambarota

Introduction: Magnetic resonance imaging (MRI) is an excellent imaging modality for soft tissues. Magnetization transfer (MT) imaging is an MRI technique that is sensitive to the bound protons of macromolecules and therefore can aid in the assessment of ne ...

2012

Role of lymphatic vessels in tumor immunity: passive conduits or active participants?

Melody Swartz

Research in lymphatic biology and cancer immunology may soon intersect as emerging evidence implicates the lymphatics in the progression of chronic inflammation and autoimmunity as well as in tumor metastasis and immune escape. Like the blood vasculature, ...

Springer Verlag2010

A non-cleavable mutant of Fas ligand does not prevent neutrophilic destruction of islet transplants

Steinunn Baekkeskov Hanahan

Background. Fas ligand (FasL) mediates apoptosis of susceptible Fas-expressing lymphocytes, and may contribute to the maintenance of peripheral tolerance. In transplantation models, however, artificial expres- sion of FasL on cellular as well as islet tran ...

Lippincott, Williams & Wilkins2000