CORRELATION OF SUBCELLULAR LOCALIZATION OF DISEASE-SPECIFIC INCLUSIONS AND SPHINGOLIPID ACTIVATOR PROTEIN-1(SAP-1)IN SULFATIDE SULFATASE-DEFICIENT FIBROBLASTS

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概要

• 論文の詳細を見る

• 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 (20 μg/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 6-7 nm periodicity. SAP-1 and specific sulfatide inclusions were simnltaneously observed in 59% of total lysosome vesicles exanined (164 out of 273) and positive staining of SAP-1 was observed in 92% of lysosomes with spcific sulfatide inclusion (164 out of 178). These results demonstraed 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.

• 日本組織細胞化学会の論文

著者

• TANIIKE Masako

  Department of Developmental Medicine (Pediatrics), Osaka University Graduate School of Medicine

• INUI Koji

  Department of Pediatrics, Osaka University Graduate School of Medicine

• Yabuuchi Hyakuji

  Department of Pediatrics, Osaka University Medical School

• Shinoda Koh

  Department Of Neuroanatomy Institute Of Higher Nervous Activity Osaka University Medical School

• Shinoda Koh

  Department Of Anatomy Yamaguchi University School Of Medicine

• Shinoda Koh

  Department Of Anatomy Kinki University School Of Medicine

• OKADA Shintaro

  Department of Pediatrics, Osaka University Medical School

• Taniike Masako

  Department Of Developmental Medicine (pediatrics) Osaka University Graduate School Of Medicine

• Shiotani Yahei

  Department of Neuroanatomy, Institute of Higher Nervous Activity Osaka University Medical School

• Shiotani Yahei

  Department Of Neuroanatomy Institute Of Higher Nervous Activity Osaka University Medical School

• Okada Shintaro

  Department Of Developmental Medicine (pediatrics) D-5 Osaka University Graduate School Of Medicine.

• Taniike Masako

  Department Of Pediatrics Osaka University Medical School

• Okada Shintaro

  Department Of Pediatrics Osaka Univer-ity Hospital

• Inui Koji

  Department Of Developmental Medicine (pediatrics) Osaka University Graduate School Of Medicine

• Okada Shintaro

  Department Of Pediatrics Osaka University

• Yabuuchi Hyakuji

  Department Of Pediatrics Osaka Univer-ity Hospital

• Yabuuchi Hyakuji

  Department Of Pediatrics Osaka University Medical School

• Inui Koji

  Inui Children's Clinic

• Taniike Masako

  Department of Child Development, United Graduate School of Child Development, Osaka University

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