Multinuclear giant cell formation mechanism of Krabbe disease patient brain

克拉伯病患者大脑多核巨细胞形成机制

• 批准号: 13470488
• 负责人: KOZUTSUMI Yasunori
• 金额: $ 8.77万
• 依托单位: Kyoto University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (B)
• 财政年份: 2001
• 资助国家: 日本
• 起止时间: 2001 至 2003
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-13470488/
• 关键词: krabbe disease; Globoid cell; multinuclear cell; psychosine; tryptophan; yeast; organelle; sphingolipid; Krabbe病; アクチン; GIoboid cells; TDAG8; multicopy library

The goal of this project is to understand the mechanism underlining multinuclear cell formation by psychosine. The following findings were obtained during 3years.(1) Multinuclear giant cell formation mechanism by PsychosinePsychosine inhibited cytokinesis and induced multinuclear cells by disrupting membrane transport to the cleavage furrow during cytokinesis.(2) Abnormal distribution of intracellular organelles by psychosine.Psychosine induced selective dispersal of lysosomes and earl endosomes without affecting the endocytosis activity of the cells.(3) Genes involved in resistance to psychosine in yeastPsychosine resistant genes were isolated using yeast system. One of genes encodes tryptophan permease. Tryptophan permease was degraded by the treatment of psychosine via the Golgi-vacuole membrane transport system.

该项目的目的是了解通过Psychosine强调多核细胞形成的机制。 （1）通过心理心理素通过精神上的多核巨细胞形成机制获得以下发现，通过破坏膜在细胞因子过程中裂解到裂解沟的裂解，抑制了细胞因子和诱导的多核细胞。 （3）使用酵母菌系统分离了抗酵母菌耐药性基因的抗抑制性的基因，而不会影响细胞的内吞作用。一个基因编码色氨酸渗透酶。色氨酸渗透酶通过Golgi-vacuole膜运输系统的心理体治疗降解。

项目成果

Ikeda et al.: "Identification and characterization of functional domains in a mixed lineage kinase LZK"FEBS Lett.. 488(3). 190-195 (2001)

Ikeda 等人：“混合谱系激酶 LZK 中功能域的识别和表征”FEBS Lett.. 488(3)。

Kasahara et al.: "Association of GPI-anchored protein TAG-1 with src-family kinase Lyn in lipid rafts of cerebellar granule cells"Neurochem Res.. 27(7-8). 823-829 (2002)

Kasahara 等人：“小脑颗粒细胞脂筏中 GPI 锚定蛋白 TAG-1 与 src 家族激酶 Lyn 的关联”Neurochem Res. 27(7-8)。

A.Ikeda et al.: "Identification and characterization of functional domains in a mixed lineage kinase LZK"FEBS Lett.. 488・3. 90-95 (2001)

A. Ikeda 等：“混合谱系激酶 LZK 中功能域的鉴定和表征” FEBS Lett.. 488・3 (2001)。

Kozutsumi et al.: "Sphingolipids involved in the induction of multinuclear cell formation"Biochim Biophys Acta. 1582(1-3). 138-143 (2002)

Kozutsumi 等人：“参与多核细胞形成诱导的鞘脂”Biochim Biophys Acta。

Kakio et al.: "Interaction between Amyloid b-Protein Aggregates and Membranes"J.Pep.Sci.. (発表予定).

Kakio 等人：“淀粉样 b-蛋白聚集体与膜之间的相互作用”J.Pep.Sci..（待出版）。