Publications associées à Small fiber peripheral neuropathy

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

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

Machine-learning predictor of nerve fiber firing rate allows the automatic optimization of electrical stimulation protocols

Silvestro Micera, Simone Romeni, Luca Pierantoni, Gabriele Marino

Computational models have been widely employed to study the electrical stimulation of the nervous system. Still, most applications either study fundamental mechanisms underlying stimulation, or address qualitative scientific questions. When quantitative qu ...

IEEE2023

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, Cédric Gobet, René Uwe Schneider

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

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, Francesco Maria Petrini, Ivo Strauss, Giacomo Valle

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, Francesca Bianchi

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

Hakan Gökcesu, Kaan Gökcesu

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, Nathalie Marie Géraldine Bernard, Cylia Cloé Rochat, Omar El Mansour, Manisha Juneja

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