Krabbe disease is an autosomal recessive inherited demyelinating disease, which 
is deficient in lysosomal enzyme, galactocerebrosidase. Pathophysiological 
characteristics of this disease are extreme demyelination in white matter and 
peripheral nerve, existence of globoid cells, absence of accumulation of main 
substrates, i.e. galactocerebrosidase in tissues and accumulation of psychosine. 
Molecular basis of this disease including isolation of a cDNA for human and 
murine galactocerebrosidase and cloning of genome of this gene are reviewed. The 
trial of gene therapy on twitcher, the mouse model of Krabbe disease, could 
break through on therapy on this progressive demyelinating disease.