Article
[Metachromatic leucodystrophy. Clinical, biological, and therapeutic aspects].
Annales de biologie clinique - 1 Jan 2000
Barboura Ilhem, Ferchichi Salima, Dandana Azza, Jaidane Zaineb, Ben Khelifa Souhaira, Chahed Hinda, Ben Mansour Rachida, Chebel Saber, Maire Irène, Miled Abdelhedi
Abstract excerpt
Scholz's disease or metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by a deficiency in arylsulfatase A (ARSA: EC 3.1.6.8). This enzyme is responsible for the degradation of sulfatides commonly called cerebroside-3-sulfate or 3-O-sulfogalactosylcéramide in galactocérébroside and sulfate. The success of hydrolysis of these sphingolipids by ARSA necessarily depends on the presence of...
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