Physiology and pathophysiology of myelinated nerve fibers.

The chief role of the axon is that of impulse conduction, which depends on the electrical cable structure and voltage-dependent ion channels of the axonal membrane. Over recent decades, the development of specialized techniques such as patch clamping and site-directed mutagenesis have established the contribution of neuronal ion channel function to the processes of impulse conduction in myelinated nerves. Recently, these insights from in vitro studies have been translated into the clinical realm. In keeping with this progress, clinical axonal excitability techniques have been developed to provide information related to the activity of a variety of ion channels, energy-dependent pumps, and ion exchange processes activated during impulse conduction in peripheral axons. These noninvasive techniques have been extensively applied to the study of the biophysical properties of human peripheral nerves in vivo and have provided important insights into axonal ion channel function in health and neurological disease, particularly in relation to the pathophysiological mechanisms that underlie neuropathy.