The effect of (+) --cyanidanol on lysosomal enzymes of I-cell fibroblasts.
(+)--Cyanidanol, a water-soluble flavonoid, when added to cultured skin fibroblasts of a patient with I-cell disease raised the intracellular concentration of beta-galactosidase but did not affect the distribution of arylsulfatase. A, alpha-mannosidase or beta-glucuronidase. The elevated accumulation of 35SO4 by I-cell, Hunter and Maroteaux-Lamy fibroblasts was decreased by the addition of (+)--cyanidanol to the culture medium, but the degradation of previously labeled, intracellular glycosaminoglycans was not. It is concluded that (+)--cyanidanol does not produce a biochemical correction of the enzymic abnormalities existing in I-cell fibroblasts.
D001578:Benzopyrans / Q000494:pharmacology*; D002392:Catechin / Q000494:pharmacology*; D002478:Cells, Cultured; D002553:Cerebroside-Sulfatase / Q000378:metabolism; D005966:Glucuronidase / Q000378:metabolism; D006801:Humans; D008247:Lysosomes / Q000201:enzymology*; D008361:Mannosidases / Q000378:metabolism; D009081:Mucolipidoses / Q000201:enzymology*; D008059:Mucopolysaccharidosis I / Q000201:enzymology; D016532:Mucopolysaccharidosis II / Q000201:enzymology; D009087:Mucopolysaccharidosis VI / Q000201:enzymology; D012867:Skin / Q000166:cytology; D001616:beta-Galactosidase / Q000378:metabolism
D001578:Benzopyrans; D002392:Catechin; D002553:Cerebroside-Sulfatase; D008361:Mannosidases; D001616:beta-Galactosidase; D005966:Glucuronidase