Charcot-Marie-Tooth disease (CMT) is the most common inherited neuromuscular disease and is characterized by

Axonal cytoskeletal and cytosolic proteins are synthesized in the neuronal cell body and transported along axons by slow axonal transport, but attempts to observe this movement directly in living cells have yielded conflicting results. Here we report the direct observation of the axonal transport of neurofilament protein tagged with green fluorescent protein in cultured nerve cells. Live-cell imaging of naturally occurring gaps in the axonal neurofilament array reveals rapid, intermittent and highly asynchronous movement of fluorescent neurofilaments. The movement is bidirectional, but predominantly anterograde. Our data indicate that the slow rate of slow axonal transport may be the result of rapid movements interrupted by prolonged pauses.

The astroglial response to CNS injury is considered in the context of neuron-glial relationships. Although previous models suggested that astroglial cells present in "scars" impede axon regrowth owing to irreversible changes in the glial cell following injury, recent in vivo and in vitro studies indicate that astroglial cells exhibit considerable plasticity, elevating expression of the glial filament protein and altering expression of properties which support axons, including extracellular matrix components and cell surface adhesion systems. Both in vivo and in vitro studies on neuron-glia interactions in different brain regions suggest that glia express region-specific properties, including ion channels, neurotransmitter uptake and receptor systems, and cell surface adhesion systems. Together these findings suggest that a more detailed analysis of glial response to injury in different brain regions will lead to an appreciation of the diversity of the astroglial response to injury, and its regulation by neuron-glia relationships.

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