title: JCL Roundtable: Hypertriglyceridemia due to defects in lipoprotein lipase function.
abstract: In this Roundtable, our intent is to discuss those rare genetic disorders that impair the function of lipoprotein lipase. These cause severe hypertriglyceridemia that appears in early childhood with Mendelian inheritance and usually with full penetrance in a recessive pattern. Dr Ira Goldberg from New York University School of Medicine and Dr Stephen Young from the University of California, Los Angeles have agreed to answer my questions about this topic. Both have done fundamental work in recent years that has markedly altered our views on lipoprotein lipase function. I am going to start by asking them to give us a brief history of this enzyme system as a clinical entity.
authors.affiliation: Emory University School of Medicine, Atlanta, GA, USA. Electronic address: editorjcl@lipid.org.
authors.forename: W Virgil
authors.initials: WV
authors.lastname: Brown
authors.affiliation: Department of Internal Medicine, NYU Langone Medical Center, New York, NY, USA.
authors.forename: Ira J
authors.initials: IJ
authors.lastname: Goldberg
authors.affiliation: Division of Cardiology, UCLA Department of Medicine, Los Angeles, CA, USA.
authors.forename: Stephen G
authors.initials: SG
authors.lastname: Young
mesh_terms: D006801:Humans; D015228:Hypertriglyceridemia / Q000201:enzymology / Q000235:genetics / Q000628:therapy; D008052:Lipid Metabolism, Inborn Errors / Q000201:enzymology / Q000235:genetics / Q000628:therapy; D008071:Lipoprotein Lipase / Q000235:genetics / Q000378:metabolism; D011173:Portraits as Topic
chemical_list: D008071:Lipoprotein Lipase
keywords: Genetic disorders; Hypertriglyceridemia; LPL; Lipase; Lipoprotein