Specific determination of arylsulfatase A activity
โ Scribed by H. Inoue; Y. Seyama; S. Yamashita
- Publisher
- Springer
- Year
- 1986
- Tongue
- English
- Weight
- 345 KB
- Volume
- 42
- Category
- Article
- ISSN
- 1420-682X
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๐ SIMILAR VOLUMES
It had been shown previously that arylsulfatase A activity was attenuated in pseudo arylsulfatase A deficiency fibroblasts and that subunits of the enzyme were smaller than subunits of the enzyme in normal fibroblasts. Attenuated enzyme activity has now been affirmed in other tissues. Subunits of th
A method for the rapid determination of the specific activity of NaB3H4 is presented. NaB3H4 is used to reduce NAD+ to [3H]NADH, which is then isolated by anion exchange chromatography. The specific activity of the NaB3H4 is calculated from measurements of radioactivity and absorbance (340 nm) in th
Deficient arylsulfatase-A activity is diagnostic of a neurodegenerative human lysosomal storage disease, metachromatic leukodystrophy. Paradoxically, similar enzyme deficiency also occurs in normal individuals, who are known as being pseudo arylsulfatase-A deficient. We showed previously that this p