title: Characterization of the cytoplasmic inclusion bodies of the spleens from patients with adult form Gaucher's disease.
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.
mesh_terms: D002784:Cholesterol / Q000032:analysis; D003593:Cytoplasm / Q000032:analysis*; D004492:Edetic Acid / Q000494:pharmacology; D005776:Gaucher Disease / Q000378:metabolism*; D006017:Glycolipids / Q000032:analysis; D006801:Humans; D002479:Inclusion Bodies / Q000032:analysis* / Q000187:drug effects / Q000648:ultrastructure; D008854:Microscopy, Electron; D010743:Phospholipids / Q000032:analysis; D011506:Proteins / Q000032:analysis; D013154:Spleen / Q000648:ultrastructure*
chemical_list: D006017:Glycolipids; D010743:Phospholipids; D011506:Proteins; D002784:Cholesterol; D004492:Edetic Acid