Autosomal Recessive Spastic Ataxia of Charlevoix‐Saguenay in Two Half‐Siblings
ABSTRACT Autosomal recessive spastic ataxia of Charlevoix‐Saguenay (ARSACS) is caused by biallelic pathogenic variants in the SACS gene. We report the clinical, radiologic and neurophysiologic features of a pair of half‐siblings who presented with progressive cerebellar ataxia, peripheral neuropathy and upper motor neuron signs.
Dennis Yeow +6 more
wiley +1 more source
Lithium promotes proliferation and suppresses migration of Schwann cells
Schwann cell proliferation, migration and remyelination of regenerating axons contribute to regeneration after peripheral nervous system injury. Lithium promotes remyelination by Schwann cells and improves peripheral nerve regeneration.
Xiao-Kun Gu +3 more
doaj +1 more source
Deletion of Tsc2 in nociceptors reduces target innervation, ion channel expression, and sensitivity to heat [PDF]
The mechanistic target of rapamycin complex 1 (mTORC1) is known to regulate cellular growth pathways, and its genetic activation is sufficient to enhance regenerative axon growth following injury to the central or peripheral nervous systems.
Carlin, Dan +6 more
core +2 more sources
Multifunctional Microstructured Surfaces by Microcontact Printing of Reactive Microgels
Reactive poly(N‐vinylcaprolactam‐co‐glycidyl methacrylate) microgels are used as functional inks to create surface‐grafted arrays on glass via microcontact printing. The patterns (10–50 µm widths and spacings) enable stable binding and post‐functionalization with dyes and peptides.
Inga Litzen +4 more
wiley +1 more source
Data on the effect of in vivo knockdown using artificial ErbB3 miRNA on Remak bundle structure
Mature Schwann cells, the peripheral nervous system (PNS) glial cells, have two major roles for neuronal axons (Bunge, 1993) [1]. For large diameter axons, Schwann cells form myelin sheaths with multiple layers.
Yuki Miyamoto +6 more
doaj +1 more source
Degeneration and regeneration of peripheral nerves: role of thrombin and its receptor PAR-1 [PDF]
The peripheral nervous system has a striking regeneration potential and after damage extensive changes in the differentiation state both of the injured neurons and of the Schwann cells are observed.
Artico, M. +7 more
core
Few‐layered three dimansional conductive graphene foams are promising cytocompatible platforms to transdifferentiate mesenchymal stem cells into Schwann cell‐like phenotypes using electrical and microstructural cues. Applied electrical stimulation conditions resulted in activation of MAPK, neurotrphin and RAS signaling pathways that led to upregulation
Ekin G. Simsar +9 more
wiley +1 more source
Administration of tumour necrosis factor α (TNFα) to axotomised mouse neonatal sciatic nerves increased Schwann cell apoptosis in the distal nerve segments, 5-fold greater than axotomy alone.
Kristy Boyle +3 more
doaj +1 more source
Peroxisomal dysfunctions cause lysosomal storage and axonal Kv1 channel redistribution in peripheral neuropathy [PDF]
Impairment of peripheral nerve function is frequent in neurometabolic diseases, but mechanistically not well understood. Here, we report a novel disease mechanism and the finding that glial lipid metabolism is critical for axon function, independent of ...
Asadollahi, Ebrahim +17 more
core +3 more sources
Decellularized Extracellular Matrix (dECM) in Tendon Regeneration: A Comprehensive Review
Decellularized Extracellular Matrix (dECM) offers a promising solution by replicating the native tendon microenvironment and promoting regeneration. This review highlights advances in the decellularization methods, as well as their integration with emerging technologies and translational progress in tendon tissue engineering.
Kumaresan Sakthiabirami +4 more
wiley +1 more source

