Molecular regulators of nerve conduction - Lessons from inherited neuropathies and rodent genetic models

Research output: Contribution to journalReview articlepeer-review

Abstract

Myelinated nerve fibers are highly compartmentalized. Helically wrapped lipoprotein membranes of myelin are integrated with subsets of proteins specifically in each compartment to shape the physiological behavior of these nerve fibers. With the advance of molecular biology and genetics, many functions of these proteins have been revealed over the past decade. In this review, we will first discuss how action potential propagation has been understood by classical electrophysiological studies. In particular, the discussion will be concentrated on how the geometric dimensions of myelinated nerve fibers (such as internodal length and myelin thickness) may affect nerve conduction velocity. This discussion will then extend into how specific myelin proteins may shape these geometric parameters, thereby regulating action potential propagation. For instance, periaxin may specifically affect the internodal length, but not other parameters. In contrast, neuregulin-1 may affect myelin thickness, but not axon diameter or internodal length. Finally, we will discuss how these basic neurobiological observations can be applied to inherited peripheral nerve diseases.

Original languageEnglish (US)
Pages (from-to)209-218
Number of pages10
JournalExperimental Neurology
Volume267
DOIs
StatePublished - May 1 2015

Keywords

  • Action potential
  • Adherens junction
  • Axonal degeneration
  • Caspr
  • Charcot-Marie-Tooth disease
  • CMT1A
  • CMT2
  • CMTX1
  • Compound muscle action potential
  • Connexin-1
  • Demyelination
  • Hereditary neuropathy with liability to pressure palsies
  • HNPP
  • Inherited neuropathy
  • Ion channel
  • Juxtaparanode
  • Myelination
  • Nerve conduction study
  • Node of Ranvier
  • Paranode
  • PMP22
  • Polyneuropathy
  • Sensory nerve action potential
  • Septate-like junction
  • Tight junction
  • Voltage-gated potassium channel
  • Voltage-gated sodium channel

ASJC Scopus subject areas

  • Neurology
  • Developmental Neuroscience

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