Krabbe's disease

náhled|Mnohojaderné makrofágy s PAS-pozitivními inkluzemi, tzv. globoidní buňky 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