CGI-58 was identified as an important factor in cellular triacylglycerol homeostasis when mutations in CGI-58 were established as the cause of Chanarin Dorfman syndrome (13), a neutral lipid storage disorder (NLSD) characterized by excessive storage of triacylglycerols in many cells and tissues (13–18). The gene discussed is ABHD5; the disease is Dorfman-Chanarin disease.