Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: EC:3.1.6.1 (
sulfatase
)
3,205
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Multiple sulfatase deficiency can be classified into group I with severe and group II with moderate deficiencies in sulfatases. In fibroblasts in both groups the stability of
arylsulfatase A
and of the 47000-Mr form of
arylsulfatase B
is decreased [F. Steckel, A. Hasilik & K. von Figura (1985) Eur. J. Biochem. 151, 141-145]. After endocytosis in control fibroblasts or those from multiple sulfatase deficiency,
arylsulfatase A
and B derived from the latter were subjected to enhanced degradation in both types of recipient cells. The degradation was closely linked in time to endocytosis. Whereas instability of
arylsulfatase A
derived from different cell lines from multiple sulfatase deficiency was comparable, a marked heterogeneity was observed for the instability of the 47000-Mr polypeptide of
arylsulfatase B
. Each of the cell lines from multiple sulfatase deficiency synthesized
arylsulfatase A
and B polypeptides with normal and with decreased stability. Treatment with benzyloxycarbonyl-Phe-Ala-
CHN2
, an inhibitor of cysteine proteinases, stabilized
arylsulfatase A
polypeptides and partially restored
arylsulfatase A
activity in group II fibroblasts. The inhibitor had no protective effect on the 47000-Mr polypeptide or the activity of
arylsulfatase B
. The bearing of these findings on the yet unknown primary defect in multiple sulfatase deficiency is discussed.
...
PMID:Multiple sulfatase deficiency: degradation of arylsulfatase A and B after endocytosis in fibroblasts. 286 39
