Krabbe disease
From WikiLectures
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.
Links[edit | edit source]
[edit | edit source]
- Leukodystrophy
- Lipidosis
- Hereditary metabolic disorders
- Hereditary small molecule metabolic disorders
- Hereditary metabolic disorders of complex molecules
Source[edit | edit 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/ >.