Some thoughts on the neurobiology of the leukodystrophies.

  title={Some thoughts on the neurobiology of the leukodystrophies.},
  author={William T. Norton},
  volume={15 Suppl},
The known leukodystrophies are genetic diseases involving defects in oligodendroglial function and myelinogenesis. The various steps in the process of myelination and myelin maintenance are outlined beginning with gliogenesis and ending with myelin catabolism. A gene defect affecting any one of these steps has the potential to lead either to a leukodystrophy or to a more systemic disease. The shiverer mouse mutant involves deletion of the gene for basic protein, and the quaking mutant may… CONTINUE READING