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Friederike Hörster

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Marina A Morath; Friederike Hörster; Sven W Sauer
Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist.
Tamaris Zwickler; Alina Riderer; Peter Burgard; Gisela Haege; Georg F Hoffmann; Friederike Hörster; Stefan Kölker
Metabolic decompensation in methylmalonic aciduria: which biochemical parameters are discriminative?
Thomas Opladen; Georg Hoffmann; Friederike Hörster; Anne-Bärbel Hinz; Katharina Neidhardt; Christine Klein; Nicole Wolf
Clinical and biochemical characterization of patients with early infantile onset of autosomal recessive GTP cyclohydrolase I deficiency without hyperphenylalaninemia.

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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: Tamaris Zwickler; Alina Riderer; Peter Burgard; Gisela Haege; Georg F Hoffmann; Friederike Hörster; Stefan Kölker
Metabolic decompensation in methylmalonic aciduria: which biochemical parameters are discriminative?
Journal of inherited metabolic disease 2012;35(5):797-806.
2011: Thomas Opladen; Georg Hoffmann; Friederike Hörster; Anne-Bärbel Hinz; Katharina Neidhardt; Christine Klein; Nicole Wolf
Clinical and biochemical characterization of patients with early infantile onset of autosomal recessive GTP cyclohydrolase I deficiency without hyperphenylalaninemia.
Movement disorders : official journal of the Movement Disorder Society 2011;26(1):157-61.
2009: Friederike Hörster; Sven F Garbade; T Zwickler; H I Aydin; Olaf Bodamer; Alberto Burlina; A M Das; J B C De Klerk; Carlo Dionisi Vici; S Geb; G Gökcay; N Guffon; Esther M Maier; Eva Morava; J H Walter; B Schwahn; F A Wijburg; Martin Lindner; S Grünewald; Matthias R Baumgartner; Stefan Kölker
Prediction of outcome in isolated methylmalonic acidurias: combined use of clinical and biochemical parameters.
Journal of inherited metabolic disease 2009;32(5):630-9.
2009: D Haas; P Niklowitz; Friederike Hörster; E Regula Baumgartner; C Prasad; Richard J T Rodenburg; Georg F Hoffmann; T Menke; Jürgen G Okun
Coenzyme Q(10) is decreased in fibroblasts of patients with methylmalonic aciduria but not in mevalonic aciduria.
Journal of inherited metabolic disease 2009;32(4):570-5.
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: Kenju Shimomura; Friederike Hörster; Heidi de Wet; Sarah Flanagan; Sian Ellard; Andrew T Hattersley; Nicole I Wolf; Frances M Ashcroft; Friedrich Ebinger
A novel mutation causing DEND syndrome: a treatable channelopathy of pancreas and brain.
Neurology 2007;69(13):1342-9.
2007: Friederike Hörster; Matthias R Baumgartner; Caroline Viardot; Terttu Suormala; Peter Burgard; Brian Fowler; Georg F Hoffmann; Sven F Garbade; Stefan Kölker; E Regula Baumgartner
Long-term outcome in methylmalonic acidurias is influenced by the underlying defect (mut0, mut-, cblA, cblB).
Pediatric research 2007;62(2):225-30.
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.
2004: Friederike Hörster; Georg F Hoffmann
Pathophysiology, diagnosis, and treatment of methylmalonic aciduria-recent advances and new challenges.
Pediatric nephrology (Berlin, Germany) 2004;19(10):1071-4.
2004: Claus Peter Schmitt; Otto Mehls; Friedrich K Trefz; Friederike Hörster; T Lutz Weber; Stefan Kölker
Reversible end-stage renal disease in an adolescent patient with methylmalonic aciduria.
Pediatric nephrology (Berlin, Germany) 2004;19(10):1182-4.
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: Stefan Kölker; Georg Köhr; Barbara Ahlemeyer; Jürgen G Okun; Verena Pawlak; Friederike Hörster; Ertan Mayatepek; Josef Krieglstein; Georg F Hoffmann
Ca(2+) and Na(+) dependence of 3-hydroxyglutarate-induced excitotoxicity in primary neuronal cultures from chick embryo telencephalons.
Pediatric research 2002;52(2):199-206.
2002: Stefan Kölker; Verena Pawlak; Barbara Ahlemeyer; Jürgen G Okun; Friederike Hörster; Ertan Mayatepek; Josef Krieglstein; Georg F Hoffmann; Georg Köhr
NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria.
The European journal of neuroscience 2002;16(1):21-8.
2002: Stefan Kölker; Jürgen G Okun; Barbara Ahlemeyer; Angela T S Wyse; Friederike Hörster; Moacir Wajner; Dirk Kohlmüller; Ertan Mayatepek; Josef Krieglstein; Georg F Hoffmann
Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons.
Journal of neuroscience research 2002;68(4):424-31.
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.
2001: Stefan Kölker; Jürgen G Okun; Friederike Hörster; Birgit Assmann; Barbara Ahlemeyer; Dirk Kohlmüller; S Exner-Camps; Ertan Mayatepek; Josef Krieglstein; Georg F Hoffmann
3-Ureidopropionate contributes to the neuropathology of 3-ureidopropionase deficiency and severe propionic aciduria: a hypothesis.
Journal of neuroscience research 2001;66(4):666-73.