Krabbe's disease

Krabbe's disease (also Krabbe's globoid leukodystrophy) belongs to the leukodystrophies. It belongs to hereditary diseases caused by congenital defect of lysosomal enzymes. The result is an unbounded, non-cancerous accumulation of mature adipose tissue in places where it does not occur under normal conditions. It is a galactocerebroside β-galactosidase deficiency. Affects CNS cells.

Links

 * ws:Krabbeho choroba

Related articles

 * Leukodystrophy
 * Lipidoses
 * Hereditary metabolic disorders
 * Inherited metabolic disorders of small molecules
 * Hereditary metabolic disorders of complex molecules