Folinic acid-responsive seizures (FARS) are a rare treatable cause of neonatal epilepsy. They have characteristic peaks on CSF monoamine metabolite analysis, and have mutations in the ALDH7A1 gene, characteristically found in pyridoxine-dependent epilepsy. There are case reports of patients presenting with seizures at a later age, and with folate deficiency due to different mechanisms with vari...

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...

1. Diets containing graded levels of pyridoxine hydrochloride (to supply 0.26--30 mg pyridoxine/kg) were given to seven duplicate groups of turbot (Scophthalmus maximus) for 12 weeks and their growth rate was measured during this period. 2. Good growth was obtained on all treatments except those groups given less than 1.0 mg pyridoxine/kg diet. These fish grew normally until weeks 8--10 but the...

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...

During studies of the nutritional utilization of pyridoxine 5*-b-D-glucoside, a major form of vitamin B6 in plants, we detected two cytosolic b-glucosidases in jejunal mucosa. As expected, one was broad specificity b-glucosidase that hydrolyzed aryl b-D-glycosides but not pyridoxine b-D-glucoside. We also found a previously unknown enzyme, designated pyridoxine-b-D-glucoside hydrolase, that eff...

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...

Dempsey, Walter B. (University of Florida, Gainesville). Control of pyridoxine biosynthesis in Escherichia coli J. Bacteriol. 90:431-437. 1965.-The total pyridoxine in a culture of exponentially growing Escherichia coli was 3.6 x 10(-10) moles per mg of dry cells. One-fourth of this total was present in the medium, and was at least 90% pyridoxal 5'-phosphate. Both pyridoxol and pyridoxal, when ...

OBJECTIVE Intractable epilepsy is a serious neurologic problem with different etiologies. Decreased levels of pyridoxal phosphate in cerebral spinal fluid of patients with intractable epilepsy due to pyridoxine dependency epilepsy are reported. The aim of this study was to compare plasma pyridoxal 5´-phosphate level in patients with intractable and controlled epilepsy. MATERIALS & METHODS Thi...