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Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A (cerebroside sulfatase) activity by inhibitors of thiol proteinases.

Arylsulfatase A polypeptides were examined in cultured fibroblasts from a patient with juvenile metachromatic leukodystrophy and three patients with the adult form of the disease, with the aid of metabolic labeling and immunoprecipitation. The mutant cells were severely deficient in the arylsulfatas...

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Vydáno v:Proc Natl Acad Sci U S A
Hlavní autoři: von Figura, K, Steckel, F, Hasilik, A
Médium: Artigo
Jazyk:Inglês
Vydáno: National Academy of Sciences 1983
Témata:
On-line přístup:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC534361/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/6136972/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.80.19.6066
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