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  • Title: Characterization of the cytoplasmic inclusion bodies of the spleens from patients with adult form Gaucher's disease.
    Author: Ebato H, Abe T, Yamakawa T, Nagashima K.
    Journal: J Biochem; 1980 Dec; 88(6):1765-72. PubMed ID: 6257671.
    Abstract:
    The twisted tubular cytoplasmic inclusion bodies were isolated from spleens of four patients with adult form Gaucher's disease. The chemical composition of the CIB (cytoplasmic inclusion bodies) was as follows: proteins, 10%, cholesterol, 10%, phospholipids, 10%, glycolipids, 70%. More than 90% of glycolipids from CIB were glucosylceramide. The structural protein profile of these bodies was examined by SDS-polyacrylamide disc gel electrophoresis on a semimicro-scale (s-PAGE). A similar protein composition which included two glycoproteins was found in all the four cases. The tubular structure of the bodies was changed to a small and round form by the treatment with 1 mM EDTA-Na2 which removed some structural proteins from the bodies. This indicated that some proteins might have an important role in maintaining the tubular structure of CIB.
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