Metachromatic leukodystrophy: Molecular genetics and an animal model

doi:10.1023/a:1005471106088

Article

Metachromatic leukodystrophy: Molecular genetics and an animal model

1 Aug 1998

Abstract excerpt

Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by the deficiency of arylsulphatase A (ASA; EC 3.1.6.8). Deficiency of this enzyme causes intralysosomal storage of the sphingolipid cerebroside sulphate. This lipid is abundant in myelin and it may thus not be surprising that storage mainly affects oligodendrocytes. Patients suffer from a progressive demyelination causing various...

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