PREPARE RADIOLABELED SPHINGOLIPIDS AND GLYCOCONJUGATES

该项目的目标包括五个主要领域。 一是 14 C-标记的葡糖脑苷脂，14 C-标记的鞘磷脂， 和14 C-标记的神经酰胺三己糖苷酶作为诊断试剂， 高雪氏症患者的识别和携带者的检测 疾病、尼曼-皮克病和法布里病。 第二、 需要这些放射性鞘脂来监测 用于临床治疗试验的酶。 第三个目标是 开发新的鞘脂，可以用荧光染料标记， 它们的细胞和亚细胞分布可以被追踪。 第四 目的是合成鞘脂的不可代谢类似物， 人脂质储存障碍的动物和组织模型。 第五 目的是合成更复杂的神经节苷脂 从而可以在正常和肿瘤组织中检测它们的代谢。

The objectives of the project encompass five principal areas. The first is the production of 14C-labeled glucocerebroside, 14C-labeled sphingomyelin, and 14C-labeled ceramidetrihexosidase for use as diagnostic reagents for the identification of patients and detection of carriers of Gaucher's disease, Niemann-Pick disease and Fabry's disease, respectively. Second, these radioactive sphingolipids are required to monitor the activity of enzymes for use in clinical therapy trials. The third objective is to develop novel sphingolipids that can be tagged with fluorescent dyes so that their cellular and subcellular distribution can be traced. The fourth objective is to synthesize non-metabolizable analogs of sphingolipids for animal and tissue models of human lipid storage disorders. The fifth objective is to synthesize more complex sphingolipids called gangliosides so that their metabolism can be examined in normal and neoplastic tissues.