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Erythrocyte antioxidant enzyme activities in children with myelomeningocele
W D Graf1, C E Pippenger, D B Shurtleff
1Department of Pediatrics, Children's Hospital and Medical Center, Seattle, WA 98105, USA.
Insights
Children with myelomeningocele and their parents often show deficiencies in antioxidant enzymes like glutathione peroxidase (GSH-Px). Lower enzyme activity may increase the risk of neural tube defects.
Area of Science:
- Biochemistry
- Pediatrics
- Genetics
Background:
- Myelomeningocele is a severe neural tube defect.
- Antioxidant enzymes play a crucial role in protecting against oxidative stress.
- Deficiencies in these enzymes may be linked to congenital anomalies.
Purpose of the Study:
- To investigate erythrocyte antioxidant enzyme activities and plasma cofactor trace elements in myelomeningocele patients.
- To compare enzyme activities between affected children, their parents, and healthy controls.
- To explore the potential link between enzyme deficiencies and neural tube defects.
Main Methods:
- Assessed erythrocyte glutathione peroxidase (GSH-Px) and glutathione reductase activities.
- Measured plasma cofactor trace elements.
- Included 26 myelomeningocele patients, 20 parents, and 14 healthy controls.
Main Results:
- Most myelomeningocele patients were deficient in erythrocyte GSH-Px.
- A high prevalence of GSH-Px deficiency was observed in parents.
- Significantly lower GSH-Px and glutathione reductase activities were found in patients and parents compared to controls.
Conclusions:
- A deficiency in antioxidant enzymes like GSH-Px is common in myelomeningocele.
- Reduced enzyme activity in parents suggests a potential genetic predisposition.
- Antioxidant enzyme deficiencies may contribute to the risk of neural tube defects.
Abstract:
Erythrocyte free radical scavenging enzyme activities and their cofactor trace elements in plasma were assessed in 26 selected patients with myelomeningocele, both parents from 10 selected families, and 14 healthy adult controls. All index children except one were deficient in erythrocyte glutathione peroxidase (GSH-Px). Nine of 10 parent pairs had at least one parent with deficient GSH-Px activity. Children with myelomeningocele had significantly lower GSH-Px activities than their parents; the group of 10 parent pairs had significantly lower GSH-Px activities than the control group; and glutathione reductase activities were significantly lower in parents and children with myelomeningocele compared with controls. A deficiency in one or more antioxidant enzymes may increase the risk for neural tube defects.