Krabbe disease (OMIM 245200), also known as globoid cell leukodystrophy (1), is an inborn error of metabolism caused by deficiency of the lysosomal enzyme β-galactocerebrosidase (GALC) (EC 3.2.1.46) (2), essential for the hydrolysis of the sphingolipid β-galactosylceramide (GalCer). Here, GALC is linked to Krabbe disease.