Available Technology

Mice with a Conditional LoxP-Flanked Glucosylceramide Synthase Allele Controlling Glycosphingolipid Synthesis

Glycosphingolipids are organizational building blocks of plasma membranes that participate in key cellular functions, such as signaling and cell-to-cell interactions. Glucosylceramide synthase - encoded by theUgcggene - controls the first committed step in the major pathway of glycosphingolipid synthesis. Global disruption of theUgcggene in mice is lethal during gastrulation. The inventors have established aUgcgallele flanked by loxP sites (floxed). When cre recombinase was expressed in the nervous system under control of thenestinpromoter, the floxed gene underwent recombination, resulting in a substantial reduction ofUgcgexpression and of glycosphingolipid ganglio-series levels. The mice deficient inUgcgexpression in the nervous system show a striking loss of Purkinje cells and abnormal neurologic sphingo-lipid behavior.
Patent Abstract: 
Glycosphingolipids are organizational building blocks of plasma membranes that participate in key cellular functions, such as signaling and cell-to-cell interactions. Glucosylceramide synthase - encoded by theUgcggene - controls the first committed step in the major pathway of glycosphingolipid synthesis. Global disruption of theUgcggene in mice is lethal during gastrulation. The inventors have established aUgcgallele flanked by loxP sites (floxed). When cre recombinase was expressed in the nervous system under control of thenestinpromoter, the floxed gene underwent recombination, resulting in a substantial reduction ofUgcgexpression and of glycosphingolipid ganglio-series levels. The mice deficient inUgcgexpression in the nervous system show a striking loss of Purkinje cells and abnormal neurologic sphingo-lipid behavior. The Research Tools available are mice with a floxedUgcgallele that can be deleted in a conditional manner. These mice carrying floxedUgcgalleles will be useful for delineating the functional roles of glycosphingolipid synthesis in the nervous system and in other physiologic systems.
applications 
Inventors: 
Richard Proia
Internal Laboratory Ref #: 
E-320-2007/0
Lab Representatives
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