Literature DB >> 2885112

A simple chromogenic assay for arylsulfatase A.

M Lee-Vaupel, E Conzelmann.   

Abstract

Arylsulfatase A hydrolyzes the artificial chromogenic substrate 4-nitrocatechol-sulfate at 0 degree C at a rate of 24% of that at 37 degrees C whereas arylsulfatase B is almost inactive at 0 degree C. Based on this observation, a simple assay was developed which permits the accurate determination of low residual arylsulfatase A activities in cultured skin fibroblasts of infantile, juvenile and adult MLD patients and pseudodeficient individuals. In cultured skin fibroblasts, the following residual activities were found with this assay system: late-infantile patients, 0.0%, one juvenile patient, 1.0%, adult patients, 4.4-14% of normal average. healthy pseudodeficient probands ranged between 18% and 32%.

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Year:  1987        PMID： 2885112     DOI： 10.1016/0009-8981(87)90068-4

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


  24 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.  External quality assurance programme for enzymatic analysis of lysosomal storage diseases: a pilot study.

Authors:  G J G Ruijter; M Boer; C W Weykamp; R de Vries; I van den Berg; J Janssens-Puister; K Niezen-Koning; R A Wevers; B J H M Poorthuis; O P van Diggelen
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.982

3.  Direct tandem mass spectrometric profiling of sulfatides in dry urinary samples for screening of metachromatic leukodystrophy.

Authors:  Ladislav Kuchař; Befekadu Asfaw; Helena Poupětová; Jitka Honzíková; František Tureček; Jana Ledvinová
Journal:  Clin Chim Acta       Date:  2013-07-06       Impact factor: 3.786

Review 4.  The anomalous kinetics of sulphatase A.

Authors:  A B Roy; T J Mantle
Journal:  Biochem J       Date:  1989-08-01       Impact factor: 3.857

5.  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

6.  Low arylsulphatase A activity and choreoathetotic syndrome in three siblings: differentiation of pseudodeficiency from metachromatic leukodystrophy.

Authors:  J Kappler; R W Watts; E Conzelmann; D A Gibbs; P Propping; V Gieselmann
Journal:  Eur J Pediatr       Date:  1991-02       Impact factor: 3.183

Review 7.  The arylsulphatase A gene and molecular genetics of metachromatic leucodystrophy.

Authors:  M L Barth; A Fensom; A Harris
Journal:  J Med Genet       Date:  1994-09       Impact factor: 6.318

8.  Compound heterozygosity for metachromatic leukodystrophy and arylsulfatase A pseudodeficiency alleles is not associated with progressive neurological disease.

Authors:  J M Penzien; J Kappler; N Herschkowitz; B Schuknecht; P Leinekugel; P Propping; T Tønnesen; H Lou; H Moser; S Zierz
Journal:  Am J Hum Genet       Date:  1993-03       Impact factor: 11.025

9.  Prevalence of common mutations in the arylsulphatase A gene in metachromatic leukodystrophy patients diagnosed in Britain.

Authors:  M L Barth; A Fensom; A Harris
Journal:  Hum Genet       Date:  1993-03       Impact factor: 4.132

10.  In vitro correction of ARSA deficiency in human skin fibroblasts from metachromatic leukodystrophy patients after treatment with microencapsulated recombinant cells.

Authors:  Valeska Lizzi Lagranha; Guilherme Baldo; Talita Giacomet de Carvalho; Maira Burin; Maria Luiza Saraiva-Pereira; Ursula Matte; Roberto Giugliani
Journal:  Metab Brain Dis       Date:  2008-09-17       Impact factor: 3.584
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