Arysulfatase A modulation with pH in multiple sulfatase deficiency disorder fibroblasts.
It has been observed that multiple sulfatase deficiency disorder (MSDD) fibroblasts contained from profoundly deficient to near normal amounts of arylsulfatase (ARS) A depending on the medium in which they were cultured. Our present findings show that the major factor determining the enzyme level is the pH of the medium during growth. In media which became acidic or was maintained at low pH (less than 7), the cells expressed the enzymopathy, while in high pH media (7.4), the cells produced enzyme. The high and low enzyme states were reversible. The ARS A deficiency in MSDD must, therefore, be a secondary manifestation of a mutation in another system.
D002553:Cerebroside-Sulfatase / Q000032:analysis* / Q000172:deficiency; D003470:Culture Media; D005347:Fibroblasts / Q000201:enzymology*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007966:Leukodystrophy, Metachromatic / Q000175:diagnosis / Q000201:enzymology*; D013429:Sulfatases / Q000032:analysis*
D003470:Culture Media; D013429:Sulfatases; D002553:Cerebroside-Sulfatase