Literature DB >> 11865283

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

Robert H J Bandsma1, Jan-Peter Rake, Gepke Visser, Richard A Neese, Marc K Hellerstein, Wim van Duyvenvoorde, Hans M G Princen, Frans Stellaard, G Peter A Smit, Folkert Kuipers.   

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.

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Year:  2002        PMID： 11865283     DOI： 10.1067/mpd.2002.121382

Source DB:  PubMed          Journal:  J Pediatr        ISSN： 0022-3476            Impact factor:   4.406


  10 in total

1.  Increased atherosclerosis in a mouse model of glycogen storage disease type 1a.

Authors:  Anouk M La Rose; Anouk G Groenen; Benedek Halmos; Venetia Bazioti; Martijn G S Rutten; Kishore A Krishnamurthy; Mirjam H Koster; Niels J Kloosterhuis; Marieke Smit; Rick Havinga; Gilles Mithieux; Fabienne Rajas; Folkert Kuipers; Maaike H Oosterveer; Marit Westerterp
Journal:  Mol Genet Metab Rep       Date:  2022-04-21

Review 2.  Understanding Inborn Errors of Metabolism through Metabolomics.

Authors:  Karen Driesen; Peter Witters
Journal:  Metabolites       Date:  2022-04-27

3.  A high activity index of stearoyl-CoA desaturase is associated with increased risk of fracture in men.

Authors:  H Melhus; U Risérus; E Warensjö; L Wernroth; K Jensevik; L Berglund; B Vessby; K Michaëlsson
Journal:  Osteoporos Int       Date:  2007-12-08       Impact factor: 4.507

4.  Glycogen Storage Disease type 1a - a secondary cause for hyperlipidemia: report of five cases.

Authors:  Patrícia Margarida Serra Carvalho; Nuno José Marques Mendes Silva; Patrícia Glória Dinis Dias; João Filipe Cordeiro Porto; Lèlita Conceição Santos; José Manuel Nascimento Costa
Journal:  J Diabetes Metab Disord       Date:  2013-06-06

5.  3-methyglutaconic aciduria in a Chinese patient with glycogen storage disease Ib.

Authors:  L K Law; N L S Tang; J Hui; C W K Lam; T F Fok
Journal:  J Inherit Metab Dis       Date:  2003       Impact factor: 4.982

6.  Skeletal muscle action of estrogen receptor α is critical for the maintenance of mitochondrial function and metabolic homeostasis in females.

Authors:  Vicent Ribas; Brian G Drew; Zhenqi Zhou; Jennifer Phun; Nareg Y Kalajian; Teo Soleymani; Pedram Daraei; Kevin Widjaja; Jonathan Wanagat; Thomas Q de Aguiar Vallim; Amy H Fluitt; Steven Bensinger; Thuc Le; Caius Radu; Julian P Whitelegge; Simon W Beaven; Peter Tontonoz; Aldons J Lusis; Brian W Parks; Laurent Vergnes; Karen Reue; Harpreet Singh; Jean C Bopassa; Ligia Toro; Enrico Stefani; Matthew J Watt; Simon Schenk; Thorbjorn Akerstrom; Meghan Kelly; Bente K Pedersen; Sylvia C Hewitt; Kenneth S Korach; Andrea L Hevener
Journal:  Sci Transl Med       Date:  2016-04-13       Impact factor: 17.956

7.  Untargeted plasma metabolomics identifies broad metabolic perturbations in glycogen storage disease type I.

Authors:  Tamara Mathis; Martin Poms; Harald Köfeler; Matthias Gautschi; Barbara Plecko; Matthias R Baumgartner; Michel Hochuli
Journal:  J Inherit Metab Dis       Date:  2021-11-10       Impact factor: 4.750

Review 8.  Flux analysis of inborn errors of metabolism.

Authors:  D-J Reijngoud
Journal:  J Inherit Metab Dis       Date:  2018-01-09       Impact factor: 4.982

9.  Impaired Very-Low-Density Lipoprotein catabolism links hypoglycemia to hypertriglyceridemia in Glycogen Storage Disease type Ia.

Authors:  Joanne A Hoogerland; Fabian Peeks; Brenda S Hijmans; Justina C Wolters; Sander Kooijman; Trijnie Bos; Aycha Bleeker; Theo H van Dijk; Henk Wolters; Albert Gerding; Karen van Eunen; Rick Havinga; Amanda C M Pronk; Patrick C N Rensen; Gilles Mithieux; Fabienne Rajas; Folkert Kuipers; Dirk-Jan Reijngoud; Terry G J Derks; Maaike H Oosterveer
Journal:  J Inherit Metab Dis       Date:  2021-04-07       Impact factor: 4.982

10.  Hepatocyte specific expression of an oncogenic variant of β-catenin results in lethal metabolic dysfunction in mice.

Authors:  Ursula J Lemberger; Claudia D Fuchs; Christian Schöfer; Andrea Bileck; Christopher Gerner; Tatjana Stojakovic; Makoto M Taketo; Michael Trauner; Gerda Egger; Christoph H Österreicher
Journal:  Oncotarget       Date:  2018-01-30
  10 in total

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