Premium
Pseudo arylsulfatase A deficiency Biosynthesis of an abnormal arylsulfatase A
Author(s) -
Ameen Mohammed,
Chang Patricia L.
Publication year - 1987
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/0014-5793(87)81204-8
Subject(s) - arylsulfatase a , arylsulfatase , metachromatic leukodystrophy , enzyme , biochemistry , biosynthesis , lysosomal storage disease , chemistry , ammonium chloride , biology , medicine , endocrinology , organic chemistry
Pseudo arylsulfatase A deficiency, an asymptomatic condition, and metachromatic leukodystrophy, a severe neurodegenerative disease, are both associated with profound reductions of arylsulfatase A activity in man. We now report that with metabolic labelling, cultured pseudo deficient cells synthesized about 20% of the normal amount of arylsulfatase A at a reduced rate of apparent synthesis and increased rate of degradation. However, in the presence of ammonium chloride which stimulated secretion of lysosomal enzymes, these cells synthesized about 80% of the normal amount of enzyme protein. Hence, the defect in pseudo arylsulfatase A deficiency is associated with labile arylsulfatase A molecules which can be stabilized if they are diverted from intracellular storage.