Literature DB >> 2889546

Assay for cerebroside sulfate (sulfatide) sulfatase in cultured skin fibroblasts with the natural activator protein.

M Lee-Vaupel1, E Conzelmann.   

Abstract

A simple procedure was developed to assay the ability of arylsulfatase A in extracts of cultured skin fibroblasts to degrade the natural substrate, sulfatide, in the presence of the physiological activator protein but without detergents. Inhibitory substances were removed by dialysis and by batch-wise ion-exchange chromatography. The enzyme recoveries during purification were monitored with a newly developed method that employs the chromogenic substrate 4-nitrocatecholsulfate at an incubation temperature of 4 degrees C. The residual sulfatidase activities determined with this procedure in fibroblasts from patients with various forms of MLD correlated well with the severity of the disease.

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Year:  1987        PMID: 2889546     DOI: 10.1016/0009-8981(87)90266-x

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN: 0009-8981            Impact factor:   3.786


  3 in total

1.  Quantitative correlation between the residual activity of beta-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease.

Authors:  P Leinekugel; S Michel; E Conzelmann; K Sandhoff
Journal:  Hum Genet       Date:  1992-03       Impact factor: 4.132

2.  Genotype-phenotype relationship in various degrees of arylsulfatase A deficiency.

Authors:  J Kappler; P Leinekugel; E Conzelmann; W J Kleijer; A Kohlschütter; T Tønnesen; M Rochel; F Freycon; P Propping
Journal:  Hum Genet       Date:  1991-03       Impact factor: 4.132

3.  Adult-onset metachromatic leucodystrophy presenting without psychiatric symptoms.

Authors:  E Klemm; E Conzelmann
Journal:  J Neurol       Date:  1989-10       Impact factor: 4.849

  3 in total

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