title: Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a.
abstract: We describe 2 patients with glycogen storage disease type 1a and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the ~40-fold increase in palmitate synthesis altering lipoprotein saturated fatty acid contents. These findings are potentially relevant for antihyperlipidemic treatment in patients with glycogen storage disease type 1a.
authors.affiliation: Groningen University Institute for Drug Exploration, Center for Liver, The Netherlands.
authors.forename: Robert H J
authors.initials: RH
authors.lastname: Bandsma
authors.forename: Jan-Peter
authors.initials: JP
authors.lastname: Rake
authors.forename: Gepke
authors.initials: G
authors.lastname: Visser
authors.forename: Richard A
authors.initials: RA
authors.lastname: Neese
authors.forename: Marc K
authors.initials: MK
authors.lastname: Hellerstein
authors.forename: Wim
authors.initials: W
authors.lastname: van Duyvenvoorde
authors.forename: Hans M G
authors.initials: HM
authors.lastname: Princen
authors.forename: Frans
authors.initials: F
authors.lastname: Stellaard
authors.forename: G Peter A
authors.initials: GP
authors.lastname: Smit
authors.forename: Folkert
authors.initials: F
authors.lastname: Kuipers
mesh_terms: D000328:Adult; D008078:Cholesterol, LDL / Q000378:metabolism*; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D006949:Hyperlipidemias / Q000150:complications*; D008079:Lipoproteins, VLDL / Q000378:metabolism; D008297:Male; D010084:Oxidation-Reduction; D014280:Triglycerides / Q000097:blood
chemical_list: D008078:Cholesterol, LDL; D008079:Lipoproteins, VLDL; D014280:Triglycerides