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Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

✍ Scribed by Astrid Potratz; Silke Hüttler; Uwe Bierfreund; Richard L. Proia; Kunihiko Suzuki; Konrad Sandhoff

Book ID: 117617756
Publisher: Elsevier Science
Year: 2000
Tongue: English
Weight: 301 KB
Volume: 1502
Category: Article
ISSN: 0925-4439
DOI: 10.1016/s0925-4439(00)00063-6

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✍ B. Schmid; B. C. Paton; K. Sandhoff; K. Harzer 📂 Article 📅 1992 🏛 Springer 🌐 English ⚖ 610 KB

Cultured skin fibroblasts from controls and patients with lysosomal storage diseases were loaded with GM1 ganglioside that had been labelled with tritium in its ceramide moiety. After a 65-h or 240-h incubation, a large percentage of this ganglioside remained undegraded in GM1 gangliosidoses, wherea