Animal models for studying the pathophysiology of ceramide

Bioactive sphingolipids play key roles in the regulation of several fundamental biological processes such as proliferation, apoptosis and transformation. The recent development of genetically engineered mouse (GEM) models has enabled the study of functional roles of sphingolipids in normal development and disease. In this chapter, we review the phenotypes of GEM models (knockout mice) that lack sphingolipid metabolism-related enzymes, discuss what we have learned from animal models and describe future directions of animal models in sphingolipid research.