CMT 2A: Critical dependence of neurons on mitochondrial dynamics

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Curr Opin Cell Biol. 2006 Jun 13

Critical dependence of neurons on mitochondrial dynamics.

Chen H, Chan DC.

Division of Biology, California Institute of Technology, 1200 East

California Blvd, MC114-96, Pasadena, CA 91125, United States.

The selective disruption of certain cell types - notably neurons - in

diseases involving mitochondrial dysfunction is thought to reflect

the high-energy requirements of these cells, but few details are

known. Recent studies have provided clues to the cellular basis of

this mitochondrial requirement.

Mitochondria are regionally organized within some nerve cells, with

higher accumulations in the soma, the hillock, the nodes of Ranvier

and the nerve terminal. In the synaptic region, mitochondria regulate

calcium and ATP levels, thereby maintaining synaptic transmission and

structure. Defects in mitochondrial dynamics can cause deficits in

mitochondrial respiration, morphology and motility.

Moreover, mutations in the mitochondrial fusion genes Mitofusin-2 and

OPA1 lead to the peripheral neuropathy Charcot-Marie-Tooth type 2A

and dominant optic atrophy. Perhaps it is the strict spatial and

functional requirements for mitochondria in neurons that cause

defects in mitochondrial fusion to manifest primarily as

neurodegenerative diseases.