Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a

Autor: Bandsma, Robert H.J., Rake, Jan-Peter, Visser, Gepke, Neese, Richard A., Hellerstein, Marc K., van Duyvenvoorde, Wim, Princen, Hans M.G., Stellaard, Frans, Smit, G.Peter A., Kuipers, Folkert
Zdroj: The Journal of Pediatrics; February 2002, Vol. 140 Issue: 2 p256-260, 5p
Abstrakt: 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. (J Pediatr 2002;140:256-60)
Databáze: Supplemental Index
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