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Brief Communication
Deficient transport of dehydroascorbic acid in the glucose transporter protein syndrome
Article first published online: 8 OCT 2004
DOI: 10.1002/ana.410440225
Copyright © 1998 American Neurological Association
1531-8249/asset/cover.gif?v=1&s=685ec69724c5ed4c8847ce939e70ceb45065856f "Annals of Neurology")
Additional Information
How to Cite
Klepper, J., Vera, J. C. and de Vivo, D. C. (1998), Deficient transport of dehydroascorbic acid in the glucose transporter protein syndrome. Ann Neurol., 44: 286–287. doi: 10.1002/ana.410440225
Publication History
- Issue published online: 8 OCT 2004
- Article first published online: 8 OCT 2004
- Manuscript Accepted: 19 MAR 1998
- Manuscript Revised: 11 MAR 1998
- Manuscript Received: 22 DEC 1997
- Abstract
- References
- Cited By
Abstract
The glucose transporter protein syndrome (GTPS) is caused by defective transport of glucose across the blood–brain barrier via the glucose transporter GLUT1, resulting in hypoglycorrhachia, infantile seizures, and developmental delay. Recent reports indicated that GLUT1 is a multifunctional transporter. We investigated the transport of vitamin C in its oxidized form (dehydroascorbic acid) via GLUT1 into erythrocytes of 2 patients with GTPS. In both patients, uptake of oxidized vitamin C was 61% of the mothers' values. Our findings are consistent with recent observations that vitamin C is transported in its oxidized form via GLUT1. We speculate that impaired transport of this substrate and perhaps other substrates in GTPS might contribute to the pathophysiology of this condition.