The assay of arylsulphatases A and B in human urine

Baum, Harold; Dodgson, K. S.; Spencer, B.

doi:10.1016/0009-8981(59)90119-6

Cited by 605 publications

(226 citation statements)

References 6 publications

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“…Rhodanese [22], arylsulfatase A (EC 3.1.6.1) according to Baurn et al [23] and glucase-6-phosphatase (EC 3.1.3.9) by the method of Swanson [24]. Mitochondria were further fractionates, according to Schnaitman and Greenawalt [25] using 0.16 mg digitonin/mg mitochondrial protein.…”

Section: Methodsmentioning

confidence: 99%

Subcellular distribution and intramitochondrial localization of three sulfurtransferases in rat liver

1975

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Section: Methodsmentioning

confidence: 99%

Subcellular distribution and intramitochondrial localization of three sulfurtransferases in rat liver

1975

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“…14 The hASA activity in tissues was evaluated by enzymatic assays using p-nitrocatecholsulfate as a substrate. 14,25 The presence of ␣-hASA antibodies in mouse serum was determined by an immunoprecipitation assay described previously. 26 Briefly, 30 l of a solution with 30 mU/ml recombinant human ASA was mixed with various dilutions of serum from transplanted mice.…”

Section: Histological Investigationsmentioning

confidence: 99%

Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy: effects on visceral and nervous system disease manifestations

et al. 2002

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“…N-acetylgalactosamine-4-sulfatase activity in leukocytes and fibroblasts was determined in vitro by spectrophotometric quantification of the p-nitrocatechol produced by hydrolysis of the substrate, p-nitrocatechol sulfate (Baum et al 1959). This assay was performed in three different samples to avoid inter-sample variation usually observed in patients.…”

Section: Biochemical and Molecular Determinationsmentioning

confidence: 99%

Clinical Evolution After Enzyme Replacement Therapy in Twins with the Severe Form of Maroteaux–Lamy Syndrome

et al. 2016

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Mucopolysaccharidosis type VI (MPS VI) is a progressive, autosomal, recessive lysosomal disorder. This disorder, due to a deficiency in N-acetylgalactosamine-4-sulfatase (ASB), results in an accumulation of glycosaminoglycan (GAG), causing multiple organ failures. In this study, monochorionic biamniotic twins with the severe form of MPS VI underwent enzyme replacement therapy (ERT) with weekly infusions of recombinant human ASB (galsulfase) at 1 mg/kg. After 9 years of ERT, a comprehensive clinical examination was performed. Several types of biochemical, immunological, and genetic investigations were also conducted. Both twins showed the typical symptoms and signs of MPS VI at baseline, including short stature, progressive dysmorphic facial features, and dysostosis multiplex. Twin 2 presented stronger multisystemic involvement, with marked musculoskeletal, neurological, and odontological components. She also developed an ischemic spinal cord lesion after surgery, which is the first case described in the literature in Maroteaux-Lamy syndrome. However, the extent of disease was found to be equally stabilized in the two sisters, concretely the cardiac and respiratory functions and body length. The early diagnosis and treatment of MPS VI are critical for an optimal clinical outcome, and further evidence for the new treatment strategies is needed.

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The assay of arylsulphatases A and B in human urine

Cited by 605 publications

References 6 publications

Subcellular distribution and intramitochondrial localization of three sulfurtransferases in rat liver

Subcellular distribution and intramitochondrial localization of three sulfurtransferases in rat liver

Bone marrow stem cell-based gene transfer in a mouse model for metachromatic leukodystrophy: effects on visceral and nervous system disease manifestations

Clinical Evolution After Enzyme Replacement Therapy in Twins with the Severe Form of Maroteaux–Lamy Syndrome

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