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Dolichol metabolism in cultured skin fibroblasts from patients with “neuronal” ceroid lipofuscinosis (Batten's disease)

✍ Scribed by B. C. Paton; A. Poulos


Publisher
Springer
Year
1984
Tongue
English
Weight
717 KB
Volume
7
Category
Article
ISSN
0141-8955

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Sphingomyelinase activity in cultured skin fibroblasts from a fetus affected with infantile-type Niemann-Pick disease was 0.5% of control activity; the activities in cells from two patients with adult-type disease (Cases 2 and 3) were 5.0% and 59.0%. Sphingomyelinase activiy was separated into three