ACTA HISTOCHEMICA ET CYTOCHEMICA
Online ISSN : 1347-5800
Print ISSN : 0044-5991
ISSN-L : 0044-5991
CORRELATION OF SUBCELLULAR LOCALIZATION OF DISEASE-SPECIFIC INCLUSIONS AND SPHINGOLIPID ACTIVATOR PROTEIN-1 (SAP-1) IN SULFATIDE SULFATASE-DEFICIENT FIBROBLASTS
MASAKO TANIIKEKOJI INUIKOH SHINODASHINTARO OKADAYAHEI SHIOTANIHYAKUJI YABUUCHI
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ジャーナル フリー

1988 年 21 巻 6 号 p. 565-573

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Sphingolipid activator protein-1 (SAP-1) stimulates enzymatic hydrolysis of sphingolipids including sulfatide. In this study, subcellular localization of the disease-specific inclusions and SAP-1 in cultured skin fibroblasts from a patient with metachromatic leukodystrophy (MLD), sulfatide storage disease, was examined by immunoelectronmicroscopy. When stained with antibody to SAP-1, acid phosphatase positive membrane-bound vesicles were stained in both the controls and MLD cells, indicating that SAP-1 was localized in lysosomes. After sulfatide loading (20μg/ml) for 7 days to MLD cells, 273 lysosomes in a cell were examined regarding the correlation of SAP-1 staining and specific sulfatide inclusions with 6-7nm periodicity. SAP-1 and specific sulfatide inclusions were simultaneously observed in 59% of total lysosome vesicles examined (164 out of 273) and positive staining of SAP-1 was observed in 92% of lysosomes with specific sulfatide inclusion (164 out of 178). These results demonstrated a positive correlation between SAP-1 staining and specific inclusions and suggested the possible induction of a secondary increase of SAP-1 in lysosomes by the pathological storage of sulfatide.
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© the Japan Society of Histochemistry and Cytochemistry
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