Mitochondrial Dynamics and Peripheral Neuropathy

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Mitochondrial Dynamics and Peripheral Neuropathy

http://nro.sagepub.com/cgi/content/abstract/14/1/12

H. Baloh

Hope Center for Neurological Disorders, Washington University, Saint

Louis, Missouri

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.