PURPOSE In infants with frequent therapy resistant seizures (TRS-infants), clinical detection of pyridoxine-dependency (PD) or -responsiveness (PR) occurs by empirical intravenous (IV) pyridoxine administration during recording of the EEG. However, in undiagnosed TRS-infants it is still unclear to what extent EEG alterations by pyridoxine-IV are attributable to PD/PR or to non-specific response...

The natural vitamin, pyridoxine, in the millimolar range is toxic to cultured rat hepatoma cells. A pyridoxine-resistant Fu5-5 rat hepatoma cell line was established by a stepwise increase in the concentration of pyridoxine in the medium. The newly established cell line, referred to as clone 10 (CI. 10), is resistant to killing by pyridoxine in concentrations up to 5 rrtM. Saturation kinetics f...

Pyridoxine-dependent epilepsy (PDE) is a rare autosomal recessive enzyme defect in the vitamin B6 metabolism characterized by intractable seizures which are usually resistant to all antiepileptic drugs but respond to pharmacological doses of pyridoxine. We present the clinical and molecular genetic findings of two patients with c.1597_1597delG mutations in ALDH7A1 gene. There are different clin...

Growth of FRM cells was inhibited by the addition of pyridoxine in a dose-dependent manner. Use of 5 mM pyridoxine caused an almost complete arrest of cell growth. Pyridoxal was as effective as pyridoxine, but pyridoxamine showed weak inhibitory action. Electron-microscopic examination of control cells revealed large nuclei and cellular membranes with villi, but, in pyridoxine-treated cells, co...

Three levels of lysine (90, 100 and 110% of Ross requirement) and of pyridoxine (3, 6 and 9 mg kg-1) were used in a 3 × 3 factorial experiment to investigate the growth and blood antioxidant ability of broilers under high ambient temperature. None of the dietary supplements affected the weight gain during the starter and grower periods. Although no significant differences were detect...

Pyridoxine (vitamin B(6)) protects neurons against neurotoxicity. An excessive release of glutamate is widely considered to be one of the molecular mechanisms of neuronal damage in several neurological diseases. We investigated whether pyridoxine affected glutamate release in rat cerebral cortex nerve terminals (synaptosomes). Pyridoxine inhibited the release of glutamate that was evoked by exp...

Pyridoxine-dependent epilepsy is a rare, autosomal recessive, treatable cause of neonatal seizures. Genetic testing can confirm mutations in the ALDH7A1 gene, which encodes antiquitin. To avoid delays in initiating treatment while awaiting confirmatory genetic testing, it is recommended that all neonates with unexplained seizures should receive trial of intravenous (IV) pyridoxine to assess for...

Wilcken, B., and Turner, B. (1973). Archives of Disease in Childhood, 48, 58. Homocystinuria: reduced folate levels during pyridoxine treatment. Nine patients with homocystinuria due to cystathionine synthase deficiency were treated with pyridoxine: 6 responded biochemically and 5 of these showed marked clinical improvement. Full biochemical response was only obtained slowly in some patients. R...

Young mice fed diets deficient in pyridoxine or fed desoxypyridoxine after the inoculation of the pneumonia virus of mice were more resistant to infection than well nourished controls. The susceptibility of young mice to PVM increased with the duration of pyridoxine administration after inoculation. Dietary protein restriction when pyridoxine was provided did not affect the susceptibility of mi...