ABSTRACT

The involvement of axonal transport in nerve and muscle diseases is of particular interest to both scientists and clinicians. The significance of abnormal axonal transport in muscular dystrophy is not clear. In addition to the mouse models for muscular dystrophy, an inherited dystrophy that affects primarily fast-twitch muscle fibers has been reported in a line of chickens. A heredity dystrophy has also been observed in Syrian hamsters, although the characteristics of this disease differ considerably from mice. A mutant mouse strain, called trembler has a Schwann cell abnormality that results in abnormal myelination patterns. Studies in experimental animals, as well as in human nerves, provide strong evidence that there is an association between axonal transport and peripheral neuropathy. It is difficult to determine whether neuropathies can arise as a consequence of abnormal axonal transport.