Disc gel electrophoresis of proteins of membranous cytoplasmic inclusion bodies from the spleen of the patient with Gaucher disease
Abe, T.; Yamakawa, T.; Endou, H.; Nagashima, K.
Japanese Journal of Experimental Medicine 48(2): 177-181
1978
ISSN/ISBN: 0021-5031 PMID: 213628 Document Number: 138720
The materials used were a normal spleen obtained from a 6-yr old child without lipid storage disease, and pathological spleen specimens from 2 patients with Gaucher disease. One case was diagnosed as infantile form of Gaucher disease and another as adult form, by clinical courses and histological studies of the spleen. The cytoplasmic inclusion bodies [CIB] were studied by both phase contrast microscopy and EM. The structural proteins of CIB from the adult form were quite different from those from the infantile form, not only qualitatively but also quantitatively. Definite differences were found in the microscale polyacrylamide disc gel electrophoresis pattern between CIB and intracellular organelles, in situ. The former contained 2 unusual glycoproteins while the latter did not.