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Trimethylaminuria, Dimethylglycine Dehydrogenase Deficiency and Disorders in the Metabolism of Glutathione

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Inborn Metabolic Diseases

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Zusammenfassung

Trimethylamine (TMA) is a volatile tertiary amine which smells of rotting fish. It is a bacterial metabolite which is produced by anaerobes resident in the human colon from choline (present in lecithin), carnitine and betaine, and from trimethylamine-N-oxide (TMAO) in salt water fish and shellfish. Catabolism of choline occurs within the mitochondria and involves the sequential removal of two methyl groups by dimethylglycine dehydrogenase (DMGDH) and sarcosine dehydrogenase (SDH). Glutathione (GSH) is a tripeptide consisting of glutamate, cysteine and glycine. It is ubiquitous in the eukaryotic organism and plays a role in many fundamental cellular processes.

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Authors and Affiliations

  1. Manchester Centre for Genomic Medicine,Willink Biochemical Genetics Unit, University of Manchester, Oxford Road, M13 9WL, Manchester, UK

    John Walter

  2. Department of Laboratory Medicine, Translational Metabolic Laboratory (830), Radboud university medical center, PO Box 9101, 6500 HB, Nijmegen, Netherlands

    Ron A. Wevers

  3. University Children’s Hospital, Heinrich-Heine-University, Moorenstraße 5, 40225, Düsseldorf, Germany

    Ertan Mayatepek

Authors
  1. John Walter
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  2. Ron A. Wevers
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  3. Ertan Mayatepek
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Corresponding authors

Correspondence to John Walter , Ron A. Wevers or Ertan Mayatepek .

Editor information

Editors and Affiliations

  1. Department of Neurology, Neurometabolic Unit, Hôpital Pitié Salpêtrière, Paris, France

    Jean-Marie Saudubray

  2. Division of Metabolism, University Children’s Hospital, Zurich, Switzerland

    Matthias R. Baumgartner

  3. Willink Biochemical Genetics Unit Manchester Centre for Genomic Medicine, University of Manchester, Manchester, UK

    John Walter

  4. Central Manchester University Hospitals NHS Foundation Trust, St Mary‘s Hospital, Manchester, UK

    John Walter

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Walter, J., Wevers, R.A., Mayatepek, E. (2016). Trimethylaminuria, Dimethylglycine Dehydrogenase Deficiency and Disorders in the Metabolism of Glutathione. In: Saudubray, JM., Baumgartner, M., Walter, J. (eds) Inborn Metabolic Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-49771-5_30

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  • DOI: https://doi.org/10.1007/978-3-662-49771-5_30

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-49769-2

  • Online ISBN: 978-3-662-49771-5

  • eBook Packages: MedicineMedicine (R0)

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