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TG hydrolysis proceeds sequentially, producing FAs and glycerol. Several reactions of CTGM can be catalyzed by more than one enzyme, creating great potential for complex tissue\u2010specific physiology. In adipose tissue, CTGM provides FA as a systemic energy source during fasting and is related to obesity. Inborn errors and mouse models have demonstrated the importance of CTGM for non\u2010adipose tissues, including skeletal muscle, myocardium and liver, because steatosis and dysfunction can occur. We discuss known inborn errors of CTGM, including deficiencies of: AGPAT2 (a form of generalized lipodystrophy), LPIN1 (childhood rhabdomyolysis), LPIN2 (an inflammatory condition, Majeed syndrome, described elsewhere in this issue), DGAT1 (protein loosing enteropathy), perilipin 1 (partial lipodystrophy), CGI\u201058 (gene <jats:italic>ABHD5<\/jats:italic>, neutral lipid storage disease (NLSD) with ichthyosis and \u201cJordan's anomaly\u201d of vacuolated polymorphonuclear leukocytes), adipose triglyceride lipase (ATGL, gene <jats:italic>PNPLA2<\/jats:italic>, NLSD with myopathy, cardiomyopathy and Jordan's anomaly), hormone\u2010sensitive lipase (HSL, gene <jats:italic>LIPE<\/jats:italic>, hypertriglyceridemia, and insulin resistance). Two inborn errors of glycerol metabolism are known: glycerol kinase (<jats:italic>GK<\/jats:italic>, causing pseudohypertriglyceridemia) and glycerol\u20103\u2010phosphate dehydrogenase (<jats:italic>GPD1<\/jats:italic>, childhood hepatic steatosis). Mouse models often resemble human phenotypes but may diverge markedly. Inborn errors have been described for less than one\u2010third of CTGM enzymes, and new phenotypes may yet be identified.<\/jats:p>","DOI":"10.1007\/s10545-014-9767-7","type":"journal-article","created":{"date-parts":[[2014,10,9]],"date-time":"2014-10-09T05:27:08Z","timestamp":1412832428000},"page":"85-98","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Inborn errors of cytoplasmic triglyceride metabolism"],"prefix":"10.1002","volume":"38","author":[{"given":"Jiang Wei","family":"Wu","sequence":"first","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Universit\u00e9 de Montr\u00e9al and CHU Sainte\u2010Justine 3175 C\u00f4te Sainte\u2010Catherine Montreal QC H3T 1C5 Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hao","family":"Yang","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Universit\u00e9 de Montr\u00e9al and CHU Sainte\u2010Justine 3175 C\u00f4te Sainte\u2010Catherine Montreal QC H3T 1C5 Canada"},{"name":"Laboratory of Animal Fat Deposition and Muscle Development, College of Animal Science and Technology Northwest A&amp;F University Yangling Shaanxi 712100 China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shu Pei","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics Universit\u00e9 de Montr\u00e9al and CHU Sainte\u2010Justine 3175 C\u00f4te Sainte\u2010Catherine Montreal QC H3T 1C5 Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krishnakant G.","family":"Soni","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Medicine, Department of Biochemistry and Biophysics Texas A&amp;M University College Station TX 77843\u20101114 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