Leukodystrophy refers to a group of disorders characterized by progressive degeneration of the
white matter of the brain. The leukodystrophies are caused by imperfect growth or development of the
myelin sheath, the fatty covering that acts as an insulator around nerve fibers. Myelin, which lends its color to the white matter of the brain, is a complex substance made up of at least ten different molecules. Each of the leukodystrophies is the result of a defect in the
gene that controls the production or metabolism of one (and only one) of the component molecules of myelin.
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Disorder of the white matter of the brain. The white matter mainly consists of nerve fibers (not the nerve cells themselves) and is concerned with conduction od nerve impulses.