Myelin lipid metabolism and its role in myelination and myelin maintenance

JA Barnes-Vélez, FBA Yasar, J Hu - The Innovation, 2023‏ - cell.com
Myelin is a specialized cell membrane indispensable for rapid nerve conduction. The high
abundance of membrane lipids is one of myelin's salient features that contribute to its unique …

Peripheral nervous system (PNS) myelin diseases

SS Scherer, J Svaren - Cold Spring Harbor Perspectives …, 2024‏ - cshperspectives.cshlp.org
This is a review of inherited and acquired causes of human demyelinating neuropathies and
a subset of disorders that affect axon–Schwann cell interactions. Nearly all inherited …

Self-assembling peptide hydrogels functionalized with LN-and BDNF-mimicking epitopes synergistically enhance peripheral nerve regeneration

S Yang, C Wang, J Zhu, C Lu, H Li, F Chen, J Lu… - …, 2020‏ - pmc.ncbi.nlm.nih.gov
The regenerative capacity of the peripheral nervous system is closely related to the role that
Schwann cells (SCs) play in construction of the basement membrane containing multiple …

PMP22 antisense oligonucleotides reverse Charcot-Marie-Tooth disease type 1A features in rodent models

HT Zhao, S Damle, K Ikeda-Lee, S Kuntz, J Li… - The Journal of clinical …, 2018‏ - jci.org
Charcot-Marie-Tooth disease type 1A (CMT1A) is caused by duplication of peripheral myelin
protein 22 (PMP22) and is the most common hereditary peripheral neuropathy. CMT1A is …

PMP22 duplication dysregulates lipid homeostasis and plasma membrane organization in develo** human Schwann cells

R Prior, A Silva, T Vangansewinkel, J Idkowiak… - Brain, 2024‏ - academic.oup.com
Abstract Charcot–Marie–Tooth disease type 1A (CMT1A) is the most common inherited
peripheral neuropathy caused by a 1.5 Mb tandem duplication of chromosome 17 …

Proteome profile of peripheral myelin in healthy mice and in a neuropathy model

SB Siems, O Jahn, MA Eichel, N Kannaiyan, LMN Wu… - Elife, 2020‏ - elifesciences.org
Proteome and transcriptome analyses aim at comprehending the molecular profiles of the
brain, its cell-types and subcellular compartments including myelin. Despite the relevance of …

Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy

R Fledrich, T Abdelaal, L Rasch, V Bansal… - Nature …, 2018‏ - nature.com
Abstract In patients with Charcot–Marie–Tooth disease 1A (CMT1A), peripheral nerves
display aberrant myelination during postnatal development, followed by slowly progressive …

Targeting PI3K/Akt/mTOR signaling in rodent models of PMP22 gene-dosage diseases

D Krauter, D Stausberg, TJ Hartmann… - EMBO Molecular …, 2024‏ - embopress.org
Haplo-insufficiency of the gene encoding the myelin protein PMP22 leads to focal myelin
overgrowth in the peripheral nervous system and hereditary neuropathy with liability to …

[HTML][HTML] Mechanisms and treatments in demyelinating CMT

V Fridman, MA Saporta - Neurotherapeutics, 2021‏ - Elsevier
Abstract Demyelinating forms of Charcot-Marie-Tooth disease (CMT) are genetically and
phenotypically heterogeneous and result from highly diverse biological mechanisms …

Wallerian demyelination: chronicle of a cellular cataclysm

N Tricaud, HT Park - Cellular and molecular life sciences, 2017‏ - Springer
Wallerian demyelination is characteristic of peripheral nerve degeneration after traumatic
injury. After axonal degeneration, the myelinated Schwann cell undergoes a stereotypical …