Gaucher氏病およびSea-blue-histiocytosisと診断された患者脾の脂質生化学的検索

概要

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Lipids of the spleen of the Gaucher's disease and that of sea-blue-histiocytosis were analysed and compared to the normal spleen. Gluco-cerebroside content in Gaucher's disease was 12.7mg/g wet weight splenic tissue and more than 50 times as much as the normal spleen. The cerebroside content in normal spleen was 0.25〜0.30mg/g wet tissue, determined by TLC-densitometry. In comparison with the normal spleen, the another neutral glycolipids, ie. lactosyl ceramide, ceramide tri-and tetra-saccharides were not accumulated in Gaucher's spleen. A GLC-analytical method on the oligo-saccharides after the ozonolysis of these glycolipids was used for the quantitative determinations. In the non-polar lipid fraction, the triglyceride content in Gaucher's disease was low (0.9μmole/g wet tissue) compared to the normal (10.9μmole/g wet tissue). And the fatty acid compositions of the gluco-cerebroside and of hematoside showed the presence of large amounts of longer chain fatty acids. The spleen of the sea-blue-histiocytosis did not show any of the accumulation of the polar and non-polar lipids. Although the Giemsa stained sea-blue-histiocytes were detected in bone-marrow, only small amounts of sea-blue-histiocytes were found in the patient's spleen. The further study on the lipids of the seabluehistiocytosis should be continued on the other tissues, such as liver, bone-marrow, etc., rather than the splenic tissue.
北里大学の論文
1974-04-30