Krabbe disease



Krabbe disease (also Krabbe globoid leukodystrophy ) is a leukodystrophy. It is one of the inherited diseases caused by a congenital defect of lysosomal enzymes. The result is an unrestricted, non-tumor accumulation of mature adipose tissue in places where it does not normally occur. This is a galactocerebroside β-galactosidase deficiency. It affects CNS cells.

related articles

 * Leukodystrophy
 * Lipidosis
 * Hereditary metabolic disorders
 * Hereditary small molecule metabolic disorders
 * Hereditary metabolic disorders of complex molecules

Source

 * VOKURKA, Martin and Jan HUGO, et al. Great Medical Dictionary [online]  . 8th edition. Prague: Maxdorf, 2009. 1144 pp. Also available from < http://lekarske.slovniky.cz/ >.