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 …

Lipids shape brain function through ion channel and receptor modulations: physiological mechanisms and clinical perspectives

S Incontro, ML Musella, M Sammari… - Physiological …, 2025‏ - journals.physiology.org
Lipids represent the most abundant molecular type in the brain, with a fat content of∼ 60%
of the dry brain weight in humans. Despite this fact, little attention has been paid to …

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 …

[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 …

Treating PMP22 gene duplication-related Charcot-Marie-Tooth disease: the past, the present and the future

S Boutary, A Echaniz-Laguna, D Adams… - Translational …, 2021‏ - Elsevier
Charcot-Marie-Tooth (CMT) disease is the most frequent inherited neuropathy, affecting
1/1500 to 1/10000. CMT1A represents 60%-70% of all CMT and is caused by a duplication …

Mechanisms and treatment strategies of demyelinating and dysmyelinating Charcot-Marie-Tooth disease

N Hertzog, C Jacob - Neural Regeneration Research, 2023‏ - journals.lww.com
Schwann cells, the myelinating glia of the peripheral nervous system, wrap axons multiple
times to build their myelin sheath. Myelin is of paramount importance for axonal integrity and …

The role of the ATP-binding cassette A1 (ABCA1) in neurological disorders: a mechanistic review

T Paseban, MS Alavi, L Etemad… - Expert Opinion on …, 2023‏ - Taylor & Francis
Introduction Cholesterol homeostasis is critical for normal brain function. It is tightly
controlled by various biological elements. ATP-binding cassette transporter A1 (ABCA1) is a …

Ion mobility–mass spectrometry reveals the role of peripheral myelin protein dimers in peripheral neuropathy

SM Fantin, KF Parson, P Yadav, B Juliano… - Proceedings of the …, 2021‏ - pnas.org
Peripheral myelin protein (PMP22) is an integral membrane protein that traffics inefficiently
even in wild-type (WT) form, with only 20% of the WT protein reaching its final plasma …

Dysregulated miR-29a-3p/PMP22 modulates Schwann cell proliferation and migration during peripheral nerve regeneration

Y Shen, Z Cheng, S Chen, Y Zhang, Q Chen… - Molecular …, 2022‏ - Springer
Schwann cells switch to a repair phenotype following peripheral nerve injury and create a
favorable microenvironment to drive nerve repair. Many microRNAs (miRNAs) are …

A novel histone deacetylase 6 inhibitor improves myelination of Schwann cells in a model of Charcot–Marie–Tooth disease type 1A

N Ha, YI Choi, N Jung, JY Song, DK Bae… - British journal of …, 2020‏ - Wiley Online Library
Background and Purpose Charcot–Marie–Tooth (CMT) disease is the most common
hereditary peripheral neuropathy. CMT type 1A (CMT1A) accounts for approximately 50% of …