Schwann cell myelination

JL Salzer - Cold Spring Harbor perspectives in biology, 2015 - cshperspectives.cshlp.org
Myelinated nerve fibers are essential for the rapid propagation of action potentials by
saltatory conduction. They form as the result of reciprocal interactions between axons and …

Disease mechanisms in inherited neuropathies

U Suter, SS Scherer - Nature reviews neuroscience, 2003 - nature.com
Inherited neuropathies are caused by dominant or recessive mutations in genes that are
expressed by neurons and/or Schwann cells. In demyelinating neuropathies, the deleterious …

[HTML][HTML] A translatable RNAi-driven gene therapy silences PMP22/Pmp22 genes and improves neuropathy in CMT1A mice

M Stavrou, A Kagiava, SG Choudury… - The Journal of …, 2022 - Am Soc Clin Investig
Charcot-Marie-Tooth disease type 1A (CMT1A), the most common inherited demyelinating
peripheral neuropathy, is caused by PMP22 gene duplication. Overexpression of WT …

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

HT Zhao, S Damle, K Ikeda-Lee… - The Journal of …, 2018 - Am Soc Clin Investig
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 …

On the molecular architecture of myelinated fibers

EJ Arroyo, SS Scherer - Histochemistry and cell biology, 2000 - Springer
Schwann cells and oligodendrocytes make the myelin sheaths of the PNS and CNS,
respectively. Their myelin sheaths are structurally similar, consisting of multiple layers of …

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 …

New evidence for secondary axonal degeneration in demyelinating neuropathies

KR Moss, TS Bopp, AE Johnson, A Höke - Neuroscience letters, 2021 - Elsevier
Abstract Development of peripheral nervous system (PNS) myelin involves a coordinated
series of events between growing axons and the Schwann cell (SC) progenitors that will …

Molecular mechanisms of inherited demyelinating neuropathies

SS Scherer, L Wrabetz - Glia, 2008 - Wiley Online Library
The past 15 years have witnessed the identification of more than 25 genes responsible for
inherited neuropathies in humans, many associated with primary alterations of the myelin …

PMP22 accumulation in aggresomes: implications for CMT1A pathology

L Notterpek, MC Ryan, AR Tobler, EM Shooter - Neurobiology of disease, 1999 - Elsevier
Peripheral myelin protein 22 (PMP22) is a 22-kDa glycoprotein mainly expressed by
Schwann cells (SCs). Duplication or deletion of the PMP22 gene locus is associated with …

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 …