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Tetrahydrobiopterin Deficiency

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Oxidative Stress and Free Radical Damage in Neurology

Abstract

Tetrahydrobiopterin has been well described as an essential cofactor for the synthesis of the neurotransmitters dopamine, norepinephrine, and serotonin. A delicate balance of tetrahydrobiopterin is also crucial for the maintenance of the oxidative environment within cells through its involvement in the nitric oxide synthase system. Failure of this system results in the production of large amounts of free radicals that serve to impair the mitochondrial electron transport chain and ultimately decrease cellular production of ATP. Tetrahydrobiopterin deficiency has been linked to many neurological disorders including atypical phenylketonuria, dystonia, Parkinson’s disease, Alzheimer’s disease, depression, and schizophrenia.

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Author information

Authors and Affiliations

  1. Department of Pediatrics, Division of Child and Adolescent Neurology, The University of Texas Health Science Center, Medical School at Houston, 6431 Fannin Street, MSB 3.153, Houston, TX, 77030, USA

    Ian J. Butler

Authors
  1. Mary Kay Koenig
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  2. Ian J. Butler
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Correspondence to Ian J. Butler .

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Editors and Affiliations

  1. Medical School, Dept. Neurology, Tel Aviv University, Tel Aviv, 69978, Israel

    Natan Gadoth

  2. Neurochirurgische Klinik, Abt. Neuropathologie, Universitätsklinikum Mainz, Langenbeckstr. 1, Mainz, 55131, Germany

    Hans Hilmar Göbel

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Koenig, M.K., Butler, I.J. (2011). Tetrahydrobiopterin Deficiency. In: Gadoth, N., Göbel, H. (eds) Oxidative Stress and Free Radical Damage in Neurology. Oxidative Stress in Applied Basic Research and Clinical Practice. Humana Press. https://doi.org/10.1007/978-1-60327-514-9_13

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