BACKGROUND Biotin-thiamine-responsive basal ganglia disease (BTRBGD) is a neurometabolic autosomal recessive (AR) disorder characterized by subacute encephalopathy with confusion, convulsions, dysarthria, and dystonia. The disease is completely reversible if treated early with biotin and thiamine, and can be fatal if left untreated.We herein present our experience with in an extended family stu...

The genes encoding thiamine kinase in Escherichia coli (ycfN) and thiamine pyrophosphokinase in Bacillus subtilis (yloS) have been identified. This study completes the identification of the thiamine salvage enzymes in bacteria.

background and purpose: mycotoxins are secondary fungal metabolites with a very high diversity that are produced by some species of aspergillus which frequently leads to contaminate food and agricultural products. recently, elimination of aflatoxin contamination in food and feed has been considered by scientists worldwide. although, the antibacterial and antifungal effects of vitamins as natura...

gerbera is one of the ten important cut flowers in terms of production and consumption in the world and iran. in this research effects of foliar application of salicylic acid and thiamine on biochemical characteristics of gerbera flower were investigated. this experiment was conducted in a completely randomized design with four replications in the greenhouse commercial of the golazin maghsoud c...

Histamine levels in thiamine deficient rats were significantly lower in the hippocampus, amygdala, olfactory bulb, thalamus and pons-medulla oblongata than those of normal and pair-fed groups. In the case of the hypothalamus, thiamine deficiency produced a significant increase in histamine levels. These changes observed in the thiamine deficient group were reversed to the normal levels by suppl...

In this issue of the Journal, Coats et al (1) describe short-term thiamine pharmacokinetics in nursing women from Cambodia who received a therapeutic course of oral thiamine for 5 d. Before supplementation, the mothers and infants were biochemically thiamine deficient. Treatment normalized thiamine status in the mothers but not in the infants, although thiamine concentrations in breast milk inc...

SYNOPSIS Analysis of five brains from patients with Leigh's disease demonstrates an accumulation of thiamine pyrophosphate and a deficiency of thiamine triphosphate. The enzyme which converts thiamine pyrophosphate to thiamine triphosphate was normally active in two of these brains, suggesting that the inhibitor found in Leigh's disease is probably producing the observed neurochemical changes. ...

In three out of five patients with low cerebrospinal fluid thiamine concentrations, the 5-hydroxyindoleacetic acid (5HIAA) values also were low. All patients received thiamine replacement therapy; they underwent a second lumbar puncture after 13, 6, 7, 5 and 45 days of treatment. In all patients blood and cerebrospinal fluid thiamine values rose after treatment. In those patients with initially...