Correction of abnormal cerebroside sulfate metabolism in cultured metachromatic leukodystrophy fibroblasts.
Cultured fibroblasts derived from patients with late infantile metachromatic leukodystrophy incorporated arylsulfatase A from the growth medium. Upon exposure to cerebroside sulfate, they exhibited patterns of uptake and hydrolysis indistinguishable from cells derived from control subjects. Furthermore, inclusion granules formed in the metachromatic leukodystrophy fibroblasts upon exposure to sulfatides were cleared by subsequent supplementation of the growth medium with arylsulfatase A.
D002554:Cerebrosides / Q000378:metabolism*; D003470:Culture Media; D046508:Culture Techniques; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000378:metabolism*; D005347:Fibroblasts / Q000378:metabolism*; D006868:Hydrolysis; D013429:Sulfatases / Q000378:metabolism; D013431:Sulfates / Q000378:metabolism*; D013460:Sulfur Isotopes
D002554:Cerebrosides; D003470:Culture Media; D013431:Sulfates; D013460:Sulfur Isotopes; D013429:Sulfatases