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  1. No Access

    Article

    Excitotoxicity and bioenergetics in glutaryl-CoA dehydrogenase deficiency

    Summary: Glutaryl-CoA dehydrogenase deficiency is an inherited organic acid disorder with predominantly neurological presentation. The biochemical hallmark of this disease is an accumulation and enhanced urinary...

    S. KÖlker, D. M. Koeller, S. Sauer, F. HÖrster… in Journal of Inherited Metabolic Disease (2004)

  2. No Access

    Article

    Emergency treatment in glutaryl-CoA dehydrogenase deficiency

    Summary: The history of glutaryl-CoA dehydrogenase deficiency is determined by acute encephalopathic crises that are precipitated by common febrile diseases, vaccinations or surgical interventions during infancy...

    S. KÖlker, C. R. Greenberg, M. Lindner, E. Müller… in Journal of Inherited Metabolic Disease (2004)

  3. No Access

    Article

    Looking forward—An evidence-based approach to glutaryl-CoA dehydrogenase deficiency

    Summary: Three decades after the first description of glutaryl-CoA dehydrogenase deficiency, major progress has been achieved in the prevention of acute striatal necrosis and neurological sequelae in affected ch...

    S. KÖlker, P. Burgard, J. G. Okun… in Journal of Inherited Metabolic Disease (2004)

  4. No Access

    Article

    Modulation of glutamatergic and GABAergic neurotransmission in glutaryl-CoA dehydrogenase deficiency

    Summary: Although the precise mechanisms underlying the CNS degeneration of patients with glutaryl-CoA dehydrogenase (GCDH) deficiency are still the subject of intense debate, many studies have highlighted that ...

    M. Wajner, S. KÖlker, D. O. Souza, G. F. Hoffmann… in Journal of Inherited Metabolic Disease (2004)

  5. No Access

    Article

    Neonatal screening for glutaryl-CoA dehydrogenase deficiency

    Summary: Acute encephalopathic crisis in glutaryl-CoA dehydrogenase deficiency results in an unfavourable disease course and poor outcome, dominated by dystonia, feeding problems, seizures and secondary complica...

    M. Lindner, S. KÖlker, A. Schulze, E. Christensen… in Journal of Inherited Metabolic Disease (2004)

  6. No Access

    Article

    Evaluation of trigger factors of acute encephalopathy in glutaric aciduria type I: Fever and tumour necrosis factor-α

    S. Kölker, B. Ahlmeyer, J. Krieglstein… in Journal of Inherited Metabolic Disease (2000)

  7. No Access

    Article

    Methylmalonic acid induces excitotoxic neuronal damage in vitro

    S. Kölker, B. Ahlmeyer, J. Krieglstein… in Journal of Inherited Metabolic Disease (2000)

  8. No Access

    Article

    Cerebral organic acid disorders induce neuronal damage via excitotoxic organic acids in vitro

    Glutaryl-CoA dehydrogenase deficiency (GDD), which is one of the most frequent organic acid disorders, is characterized by a specific age- and regional-dependent neuropathology. We hypothesized that the disti...

    S. Kölker, B. Ahlemeyer, J. Krieglstein, G. F. Hoffmann in Amino Acids (2000)

  9. No Access

    Article

    3-Hydroxyglutaric and glutaric acids are neurotoxic through NMDA receptors in vitro

    S. Kölker, B. Ahlemeyer, J. Krieglstein… in Journal of Inherited Metabolic Disease (1999)

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