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Krabbe disease (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare, often fatal degenerative disorder that affects the myelin sheath of the nervous system.It is a form of sphingolipidosis, as it involves dysfunctional metabolism of sphingolipids.he disease is named after the Danish neurologist Knud Haraldsen Krabbe (1885-1965). Illinois, Kentucky and Pennsylvania have laws to test for Krabbe and other diseases at birth. There is a movement in Maine and other states to adopt such regulations.
Mutations in the GALC gene cause Krabbe disease. These mutations cause a deficiency of the enzyme galactosylceramidase. This deficiency leads to a progressive loss of myelin that covers many nerves.rabbe disease is part of a group of disorders known as leukodystrophies, which result from the loss of myelin (demyelination). This disorder is also characterized by the abnormal presence of globoid cells, which are globe-shaped cells that usually have more than one nucleus.