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Sven Sauer

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Marina A Morath; Friederike Hörster; Sven W Sauer
Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist.
Marcin Kaminski; Peter H Krammer; Silvana Opp; Thorsten Ruppert; Sven W Sauer; Paulius Grigaravičius; Hermann-Josef Gröne; Przemysław Grudnik; Marian Kamiński; Karsten Gülow
T cell activation is driven by an ADP-dependent glucokinase linking enhanced glycolysis with mitochondrial reactive oxygen species generation.
Sven W Sauer; Silvana Opp; Georg F Hoffmann; David M Koeller; Jürgen G Okun; Stefan Kölker
Therapeutic modulation of cerebral L-lysine metabolism in a mouse model for glutaric aciduria type I.

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All Publications

2013: Marina A Morath; Friederike Hörster; Sven W Sauer
Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist.
Pediatric nephrology (Berlin, Germany) 2013;28(2):227-35.
2012: Marcin Kaminski; Peter H Krammer; Silvana Opp; Thorsten Ruppert; Sven W Sauer; Paulius Grigaravičius; Hermann-Josef Gröne; Przemysław Grudnik; Marian Kamiński; Karsten Gülow
T cell activation is driven by an ADP-dependent glucokinase linking enhanced glycolysis with mitochondrial reactive oxygen species generation.
Cell reports 2012;2(5):1300-15.
2011: Sven W Sauer; Silvana Opp; Georg F Hoffmann; David M Koeller; Jürgen G Okun; Stefan Kölker
Therapeutic modulation of cerebral L-lysine metabolism in a mouse model for glutaric aciduria type I.
Brain : a journal of neurology 2011;134(Pt 1):157-70.
2010: Sven W Sauer; Silvana Opp; Anne Mahringer; Marcin Kaminski; Christian Thiel; Jürgen G Okun; Gert Fricker; Marina A Morath; Stefan Kölker
Glutaric aciduria type I and methylmalonic aciduria: simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood-brain barrier and the choroid plexus.
Biochimica et biophysica acta 2010;1802(6):552-60.
2009: Sven W Sauer; S Opp; A Haarmann; Jürgen G Okun; Stefan Kölker; M A Morath
Long-term exposure of human proximal tubule cells to hydroxycobalamin[c-lactam] as a possible model to study renal disease in methylmalonic acidurias.
Journal of inherited metabolic disease 2009;32(6):720-7.
2008: Sven W Sauer; Jürgen G Okun; Georg F Hoffmann; Stefan Koelker; Marina A Morath
Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism.
Biochimica et biophysica acta 2008;1777(10):1276-82.
2008: Britta Keyser; Markus Glatzel; Franziska Stellmer; Bastian Kortmann; Zoltan Lukacs; Stefan Kölker; Sven W Sauer; Nicole Muschol; Wilhelm Herdering; Joachim Thiem; Stephen I Goodman; David M Koeller; Kurt Ullrich; Thomas Braulke; Chris Mühlhausen
Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1.
Biochimica et biophysica acta 2008;1782(6):385-90.
2008: Stefan Kölker; M A Morath; I Müller; Georg F Hoffmann; Sven W Sauer; Jürgen G Okun
Pathogenesis of CNS involvement in disorders of amino and organic acid metabolism.
Journal of inherited metabolic disease 2008;31(2):194-204.
2008: M A Morath; Jürgen G Okun; I B Müller; Sven W Sauer; Friederike Hörster; Georg F Hoffmann; Stefan Kölker
Neurodegeneration and chronic renal failure in methylmalonic aciduria--a pathophysiological approach.
Journal of inherited metabolic disease 2008;31(1):35-43.
2007: Sven W Sauer
Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency.
Journal of inherited metabolic disease 2007;30(5):673-80.
2007: Sven W Sauer; Stefan Kölker; Georg F Hoffmann; Herman J Ten Brink; Cornelis Jakobs; K Michael Gibson; Jürgen G Okun
Enzymatic and metabolic evidence for a region specific mitochondrial dysfunction in brains of murine succinic semialdehyde dehydrogenase deficiency (Aldh5a1-/- mice).
Neurochemistry international 2007;50(4):653-9.
2006: Marina A Schwab; Sven W Sauer; Jürgen G Okun; Leo G J Nijtmans; Richard J T Rodenburg; Lambert P van den Heuvel; Stefan Dröse; Ulrich Brandt; Georg F Hoffmann; Henk ter Laak; Stefan Kölker; Jan A M Smeitink
Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.
The Biochemical journal 2006;398(1):107-12.
2006: Stefan Kölker; Sven W Sauer; Jürgen G Okun; Georg F Hoffmann; David M Koeller
Lysine intake and neurotoxicity in glutaric aciduria type I: towards a rationale for therapy?
Brain : a journal of neurology 2006;129(Pt 8):e54.
2006: Sven W Sauer; Jürgen G Okun; Gert Fricker; Anne Mahringer; Ines Müller; Linda R Crnic; Chris Mühlhausen; Georg F Hoffmann; Friederike Hörster; Stephen I Goodman; Cary O Harding; David M Koeller; Stefan Kölker
Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency.
Journal of neurochemistry 2006;97(3):899-910.
2006: Friederike Hörster; Marina A Schwab; Sven W Sauer; Joachim Pietz; Georg F Hoffmann; Jürgen G Okun; Stefan Kölker; Stefan Kins
Phenylalanine reduces synaptic density in mixed cortical cultures from mice.
Pediatric research 2006;59(4 Pt 1):544-8.
2005: Sven W Sauer; Jürgen G Okun; Marina A Schwab; Linda R Crnic; Georg F Hoffmann; Stephen I Goodman; David M Koeller; Stefan Kölker
Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A.
The Journal of biological chemistry 2005;280(23):21830-6.
2005: Marina A Schwab; Stefan Kölker; Lambert P van den Heuvel; Sven W Sauer; Nicole I Wolf; Dietz Rating; Georg F Hoffmann; Jan A M Smeitink; Jürgen G Okun
Optimized spectrophotometric assay for the completely activated pyruvate dehydrogenase complex in fibroblasts.
Clinical chemistry 2005;51(1):151-60.
2004: Jürgen G Okun; Sven W Sauer; S Bähr; H Lenhartz; Ertan Mayatepek
S-Acetylglutathione normalizes intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency.
Journal of inherited metabolic disease 2004;27(6):783-6.
2004: David M Koeller; Sven W Sauer; Moacir Wajner; CF de Mello; Stephen I Goodman; Michael Woontner; Chris Mühlhausen; Jürgen G Okun; Stefan Kölker
Animal models for glutaryl-CoA dehydrogenase deficiency.
Journal of inherited metabolic disease 2004;27(6):813-8.
2004: Stefan Kölker; David M Koeller; Sven W Sauer; Friederike Hörster; Marina A Schwab; Georg F Hoffmann; Kurt Ullrich; Jürgen G Okun
Excitotoxicity and bioenergetics in glutaryl-CoA dehydrogenase deficiency.
Journal of inherited metabolic disease 2004;27(6):805-12.
2003: Stefan Kölker; Marina Schwab; Friederike Hörster; Sven W Sauer; Angela Hinz; Nicole I Wolf; Ertan Mayatepek; Georg F Hoffmann; Jan A M Smeitink; Jürgen G Okun
Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain.
The Journal of biological chemistry 2003;278(48):47388-93.
2002: Jürgen G Okun; Friederike Hörster; Lilla M Farkas; Patrik Feyh; Angela Hinz; Sven W Sauer; Georg F Hoffmann; Klaus Unsicker; Ertan Mayatepek; Stefan Kölker
Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity.
The Journal of biological chemistry 2002;277(17):14674-80.