PREPARE RADIOLABELED SPHINGOLIPIDS AND GLYCOCONJUGATES

该项目的目标包括五个主要领域。第一个是 ~(14)C标记的葡萄糖脑苷、~(14)C标记的鞘磷脂的生产 和14C标记的神经酰胺三己糖苷酶用作诊断试剂 高雪氏病患者的识别和携带者的检测 疾病、Niemann-Pick病和Fabry病。第二, 这些放射性鞘磷脂被要求用来监测 用于临床治疗试验的酶。第三个目标是 开发可用荧光染料标记的新型鞘脂 它们的细胞和亚细胞分布是可以追踪的。第四次 目的是合成非代谢的鞘磷脂类似物，用于 人类脂肪储存障碍的动物和组织模型。五号 目的是合成更复杂的神经鞘糖脂，称为神经节苷脂 这样就可以在正常组织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.