Mitochondrial Dynamics and Peripheral Neuropathy

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Neuroscientist. 2007 Oct 2

Mitochondrial Dynamics and Peripheral Neuropathy.

Baloh RH.

Department of Neurology, Neuromuscular Division, Washington

University.

Peripheral neuropathy is perhaps the archetypal disease of axonal

degeneration, characteristically involving degeneration of the

longest axons in the body. Evidence from both inherited and acquired

forms of peripheral neuropathy strongly supports that the primary

pathology is in the axons themselves and points to disruption of

axonal transport as an important disease mechanism. Recent studies

in human genetics have further identified abnormalities in

mitochondrial dynamics-the fusion, fission, and movement of

mitochondria- as a player in the pathogenesis of inherited

peripheral neuropathy. This review provides an update on the

mechanisms of mitochondrial trafficking in axons and the emerging

relationship between the disruption of mitochondrial dynamics and

axonal degeneration. Evidence suggests mitochondria are a " critical

cargo " whose transport is necessary for proper axonal and synaptic

function. Importantly, understanding the regulation of mitochondrial

movement and the consequences of decreased axonal mitochondrial

function may define new paths for therapeutic agents in peripheral

neuropathy and other neurodegenerative diseases.