Three-dimensional imaging of cells and tissues for the clarification of pathogenesis of inherited metabolic diseases.
细胞和组织的三维成像,用于阐明遗传性代谢疾病的发病机制。
基本信息
- 批准号:06670847
- 负责人:
- 金额:$ 1.34万
- 依托单位:
- 依托单位国家:日本
- 项目类别:Grant-in-Aid for General Scientific Research (C)
- 财政年份:1994
- 资助国家:日本
- 起止时间:1994 至 1995
- 项目状态:已结题
- 来源:
- 关键词:
项目摘要
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.
对鞘脂病的基本了解需要临床、生化和病理方面的研究。为了研究病理学方面,必须观察受疾病影响的器官和组织。我们使用体积可视化技术来创建一个受晚期婴儿异染性脑白质营养不良(MLD)影响的大脑和一个受法布里病影响的肾活检组织的三维(3D)图像。MLD患者的3D大脑图像清晰、立体、非侵入性地显示了脑内病变。该病变在磁共振(MR)图像中显示为高信号,并延伸至整个脑室周围白色物质。所提供的3D脑图像结合二维MR图像整合信息。体积射线投射是有用的,直接获得整个大脑的图像,并允许直观地了解病变的三维扩展和MLD大脑中的缺陷的程度。等值面便于明确提取病变位于体积射线投射。本研究中使用的每种技术在可视化方面都发挥了作用,它们的使用是互补的。激光扫描共聚焦显微镜分析和体积可视化的结合立体地显示了法布里病患者活检肾组织中神经酰胺三己糖苷的积聚。3D图像将促进鞘脂病的基础和临床研究。
项目成果
期刊论文数量(107)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
Kuroki, Y.: "A nobel missense mutation(C552Y) is present in the β-hexosaminidase β-subunit gene of a Japanese patient with infantile Sandhoff disease." Biochem. Biophys. Res. Commun.212. 564-571 (1995)
Kuroki, Y.:“患有婴儿桑霍夫病的日本患者的 β-己糖胺酶 β 亚基基因中存在诺贝尔错义突变 (C552Y)。Biochem。212。Commun。”
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- 影响因子:0
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Takenaka, T.: "Coexistence of gene mutations causing Fabry Disease and Duchenne muscular dystrophy in a Japanese boy." Clin. Genet.(in press).
Takenaka, T.:“导致日本男孩法布里病和杜氏肌营养不良症的基因突变共存。”
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- 影响因子:0
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Ishii, N., et al.: "Normal serum beta-galactosidase in juvenile GM1 gangliosidosis." Pediatr.Neurol.10. 317-319 (1994)
Ishii, N. 等人:“幼年 GM1 神经节苷脂沉积症中的正常血清 β-半乳糖苷酶。”
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- 影响因子:0
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Itoh, K., et al.: "Immunofluorescence analysis of globotriaosylceramide accumulated in the hearts of variant hemizygotes and heterzygotes with Fabry disease." Am.J.Cardiol. (in press).
Itoh, K. 等人:“对患有法布里病的变异半合子和杂合子心脏中积累的三酰神经酰胺进行免疫荧光分析。”
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- 影响因子:0
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- 通讯作者:
Okumiya T, et al: "α-Galactosidase gene mutations in Fabry disease: heterogeneous expressions of mutant enzyme proteins." Hum Genet. 95. 557-561 (1995)
Okumiya T 等人:“法布里病中的 α-半乳糖苷酶基因突变:突变酶蛋白的异质表达。”Hum Genet。
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- 影响因子:0
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SAKURABA Hitoshi其他文献
ハニカム構造フィルム上におけるフィブロネクチンの吸着構造と細胞接着
蜂窝结构膜上的纤连蛋白吸附结构和细胞粘附
- DOI:
- 发表时间:
2006 - 期刊:
- 影响因子:0
- 作者:
V Laquintana;N Denora;A Lopedota;H Suzuki;M Sawada;M Serra;G Biggio A Latrofa;G Trapani;G Liso;IMAI Fumihiro;NAGATSU Toshiharu;SAKURABA Hitoshi;HAYASHI Yoshinori;Yamada Jun;S. Yamamoto,;ITO Sachiko;S. Yamamoto;SAWADA Makoto;NAGATSU Toshiharu;山本貞明;S.Yamamoto;S.Yamamoto;澤田 誠;A.Tsuruma;SAWADA Makoto;山本貞明 - 通讯作者:
山本貞明
SAKURABA Hitoshi的其他文献
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{{ truncateString('SAKURABA Hitoshi', 18)}}的其他基金
Development of a new biomarker of GM2 gangliosidosis
GM2 神经节苷脂沉积症新生物标志物的开发
- 批准号:
23659527 - 财政年份:2011
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for Challenging Exploratory Research
Thermodynamic and structural study on the interaction of an enzyme and a substrate analogue for development of new therapy for lysosomal diseases
酶与底物类似物相互作用的热力学和结构研究,用于开发溶酶体疾病新疗法
- 批准号:
21390314 - 财政年份:2009
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for Scientific Research (B)
Structure-based modification of lysosomal enzymes: development of new enzyme replacement therapy for lysosomal diseases
基于结构的溶酶体酶修饰:开发溶酶体疾病的新酶替代疗法
- 批准号:
18390303 - 财政年份:2006
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for Scientific Research (B)
Development of enzyme replacement therapy for lysosomal diseases using yeast expression system.
使用酵母表达系统开发溶酶体疾病的酶替代疗法。
- 批准号:
15591149 - 财政年份:2003
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for Scientific Research (C)
Metabolism of lysosomal sialidase and molecular basis of sialidosis
溶酶体唾液酸酶的代谢和唾液酸贮积症的分子基础
- 批准号:
12670801 - 财政年份:2000
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for Scientific Research (C)
Structural analysis and molecular designing of enzyme proteins : Its application to clarification of pathology of inherited metabolic diseases and development of therapy
酶蛋白的结构分析和分子设计:其在遗传性代谢疾病病理学阐明和治疗开发中的应用
- 批准号:
08670932 - 财政年份:1996
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for Scientific Research (C)
Gene expression and its application to the investigation on pathogenesis of congenital metabolic diseases and development of therapy for them.
基因表达及其在先天性代谢性疾病发病机制研究和治疗开发中的应用。
- 批准号:
03670516 - 财政年份:1991
- 资助金额:
$ 1.34万 - 项目类别:
Grant-in-Aid for General Scientific Research (C)
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8521564 - 财政年份:2012
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Metachromatic Leukodystrophy Enzyme Drug Development
异染性脑白质营养不良酶药物开发
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8390170 - 财政年份:2012
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ASYMPTOMATIC METACHROMATIC LEUKODYSTROPHY SCREENING
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2039189 - 财政年份:1997
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ASYMPTOMATIC METACHROMATIC LEUKODYSTROPHY SCREENING
无症状异染性脑白质营养不良筛查
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2892259 - 财政年份:1997
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