PMID: 2498072May 1, 1989Paper

Pyruvate metabolism in Lafora disease

Epilepsia
H L BusardK J Lamers

Abstract

Lafora disease is an autosomal recessive and progressive degenerative disorder of the central nervous system (CNS). The pathogenic mechanism has been presumed to be an inborn error of carbohydrate metabolism, although this has never been proved. In a case of proven Lafora disease, pyruvate metabolism, which has a central position in carbohydrate metabolism, was studied in body fluids under various conditions and in brain biopsy material. No abnormalities in this metabolic pathway were found. This finding plus earlier reports in the literature exclude a defect in glycolysis; thus, a disturbance of carbohydrate metabolism as the pathogenic mechanism of Lafora disease is unlikely.

References

Jun 5, 1976·Lancet·J P BlassH G Dunn
Jan 1, 1979·Neurology·R A Kark, M Rodriguez-Budelli
Aug 4, 1975·Clinica Chimica Acta; International Journal of Clinical Chemistry·S YokoiT Negishi
Dec 30, 1975·Acta Neuropathologica·S YokoiS Maeda
Nov 1, 1979·Neurology·L L Williams
Aug 14, 1987·Clinica Chimica Acta; International Journal of Clinical Chemistry·K J LamersW O Renier
Feb 1, 1987·Journal of the Neurological Sciences·P M Van ErvenK J Lamers
Jan 15, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·K J LamersW A Lemmens
Oct 31, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·K J LamersW O Renier
Dec 1, 1974·Archives of Neurology·D L ColemanR E Blume
Mar 1, 1970·The Journal of Clinical Investigation·J P BlassB W Uhlendorf
Sep 1, 1984·Acta Neurologica Scandinavica·F J GabreëlsJ L Slooff
Apr 1, 1981·Neurology·M PrickJ Kok

❮ Previous
Next ❯

Related Concepts

Related Feeds

CSF & Lymphatic System

This feed focuses on Cerebral Spinal Fluid (CSF) and the lymphatic system. Discover the latest papers using imaging techniques to track CSF outflow into the lymphatic system in animal models.