Characterization of the cytoplasmic inclusion bodies of the spleens from patients with adult form Gaucher's disease.
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.
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*
D006017:Glycolipids; D010743:Phospholipids; D011506:Proteins; D002784:Cholesterol; D004492:Edetic Acid