| Project/Area Number |
06670847
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | The Tokyo Metropolitan Institute of Medical Science |
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Principal Investigator |
SAKURABA Hitoshi The Tokyo Metropolitan Institute of Medical Science, Department of Clinical Genetics, Research Scientist, 臨床遺伝学研究部門, 研究員 (60114493)
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| Co-Investigator(Kenkyū-buntansha) |
FUJISHIRO Issei Ochanomizu University, Department of Information Sciences, Faculty of Science, A, 理学情報科学科, 助教授 (00181347)
KOTANI Masaharu The Tokyo Metropolitan Institute of Medical Science, Department of Tumor Immunol, 臨床遺伝学研究部門, 研究員 (10195737)
ITOH Kohji The Tokyo Metropolitan Institute of Medical Science, Department of Clinical Gene, 臨床遺伝学研究部門, 研究員 (00184656)
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| Project Period (FY) |
1994 – 1995
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| Project Status |
Completed (Fiscal Year 1995)
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| Budget Amount *help |
¥2,100,000 (Direct Cost: ¥2,100,000)
Fiscal Year 1995: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1994: ¥1,400,000 (Direct Cost: ¥1,400,000)
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| Keywords | Lipidosis / Metachromatic leukodystrophy / Fabry disease / Volume visualization / Three-dimensional images / confocal laser scanning microscopy / 副腎白質ジストロフィー / レーザー走査型共焦点顕微鏡 / ボリュームビジュアライゼーション / 異染性脳白質ジストロフィー / 脳MRI |
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| Research Abstract |
The basic understanding of sphingolipidosis requires study of the clinical, biochemical, and pathological aspects. To study the pathological aspects, the organs and tissues affected by the disease must be observed. We have used volume visualization techniques to create three-dimensional (3D) images of a brain affected by late infantile metachromatic leukodystrophy (MLD) and of a biopsied kidney tissue affected by Fabry disease.
The 3D brain images of a MLD patient showed clearly, stereographically, and non-invasively the intracerebral lesion. This lesion, which indicated hyperintensity in magnetic resonance (MR) images, extended throughout the periventricular white matter. The 3D brain images provided to integrate information in combination with two-dimensional MR images. Volumetric ray-casting was useful in obtaining directly images of the entire brain and in allowing an intuitive understanding of the extension of the lesion in three dimensions and of the extent of the defects in the MLD brain. Isosurfacing facilitated a clear extraction of the lesion located by volumetric ray-casting. Each technique used in this study playd a role in visualization and their use was complementary.
The combination of a laser scanning confocal microscopic analysis and the volume visualization showed stereographically the accumulation of globotriaosylceramide in the biopsied kidney tissue from a patient with Fabry disease.
3D images will promote basic and clinical investigations of sphingolipidosis.
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