Tetrahydrobiopterin in antenatal brain hypoxia-ischemia-induced motor impairments and cerebral palsy

Redox Biol. 2017 Oct:13:594-599. doi: 10.1016/j.redox.2017.08.002. Epub 2017 Aug 3.

Abstract

Antenatal brain hypoxia-ischemia, which occurs in cerebral palsy, is considered a significant cause of motor impairments in children. The mechanisms by which antenatal hypoxia-ischemia causes brain injury and motor deficits still need to be elucidated. Tetrahydrobiopterin is an important enzyme cofactor that is necessary to produce neurotransmitters and to maintain the redox status of the brain. A genetic deficiency of this cofactor from mutations of biosynthetic or recycling enzymes is a well-recognized factor in the development of childhood neurological disorders characterized by motor impairments, developmental delay, and encephalopathy. Experimental hypoxia-ischemia causes a decline in the availability of tetrahydrobiopterin in the immature brain. This decline coincides with the loss of brain function, suggesting this occurrence contributes to neuronal dysfunction and motor impairments. One possible mechanism linking tetrahydrobiopterin deficiency, hypoxia-ischemia, and neuronal injury is oxidative injury. Evidence of the central role of the developmental biology of tetrahydrobiopterin in response to hypoxic ischemic brain injury, especially the development of motor deficits, is discussed.

Keywords: Dopamine; Fetal brain MRI; Hypertonia; Neuronal nitric oxide synthase; Reactive oxygen species; Sepiapterin.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Alcohol Oxidoreductases / deficiency
  • Alcohol Oxidoreductases / genetics
  • Alcohol Oxidoreductases / metabolism
  • Animals
  • Biopterins / analogs & derivatives*
  • Biopterins / genetics
  • Biopterins / metabolism
  • Cerebral Palsy / etiology
  • Cerebral Palsy / genetics
  • Cerebral Palsy / metabolism*
  • GTP Cyclohydrolase / deficiency
  • GTP Cyclohydrolase / genetics
  • GTP Cyclohydrolase / metabolism
  • Humans
  • Hypoxia-Ischemia, Brain / complications
  • Hypoxia-Ischemia, Brain / genetics
  • Hypoxia-Ischemia, Brain / metabolism*
  • Oxidoreductases / deficiency
  • Oxidoreductases / genetics
  • Oxidoreductases / metabolism
  • Phosphorus-Oxygen Lyases / deficiency
  • Phosphorus-Oxygen Lyases / genetics
  • Phosphorus-Oxygen Lyases / metabolism

Substances

  • Biopterins
  • Oxidoreductases
  • quinonoid dihydropterin reductase
  • Alcohol Oxidoreductases
  • sepiapterin reductase
  • GTP Cyclohydrolase
  • Phosphorus-Oxygen Lyases
  • 6-pyruvoyltetrahydropterin synthase
  • sapropterin