Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a.
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.
Bandsma
Robert H J
RH
Groningen University Institute for Drug Exploration, Center for Liver, The Netherlands.
Rake
Jan-Peter
JP
Visser
Gepke
G
Neese
Richard A
RA
Hellerstein
Marc K
MK
van Duyvenvoorde
Wim
W
Princen
Hans M G
HM
Stellaard
Frans
F
Smit
G Peter A
GP
Kuipers
Folkert
F
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
D008078:Cholesterol, LDL; D008079:Lipoproteins, VLDL; D014280:Triglycerides