Results Since 2006, 281 patients (1-73 year old) were included in the Wilson France database (sex ratio: 1). Mean age at diagnosis was 19 years. First symptoms were neurological for 36% of the patients, hepatic for 38%, renal, psychiatric or hematologic for 11%. Fifteen percent were diagnosed after familial screening. At time of diagnosis, Kayser-Fleischer ring was observed in 95% of patients w...

There is a monography of Triethylenetetramine dichlorhydrate (Trientine) in the United States Pharmacopeia. But neither the base nor the salts di- or tetra-chlorhydrate are in the European Pharmacopeia. Triethylène tetramine tetrachlorhydrate, used by AGEPS now as matural, is more soluble then triethylene tetramine dichlorhydrate. It is administred to patients with Wilson's disease, which resul...

Copper, an essential trace element acquired through nutrition, is an important co-factor for pro-angiogenic factors including vascular endothelial growth factor (VEGF). Decreasing bioavailable copper has been used as an anti-angiogenic and anti-cancer strategy with promising results. However, the role of copper and its potential as a therapy in mesothelioma is not yet well understood. Therefore...

To determine whether microbial chemosensors can be used to find new or better biocatalysts, we constructed Escherichia coli hosts that recognize the product of a biocatalytic conversion through the transcriptional activator NahR and respond by expression of a lacZ or tetA reporter gene. Equipped with a benzaldehyde dehydrogenase (XylC from Pseudomonas putida), the lacZ-based host responded to t...

Metal catalyzed oxidation via the oxidative system Cu(2+)/ascorbate is known to induce aggregation of therapeutic proteins, resulting in enhanced immunogenicity. Hence, inclusion of antioxidants in protein formulations is of great interest. In this study, using recombinant human insulin (insulin) as a model, we investigated the ability of several excipients, in particular triethylenetetramine (...

LL present theories of crossing over tacitly assume that events in one tetA rad (at least as far as crossing over is concerned) are independent of those occurring in the other tetrads of the cell. Yet as long ago as 1919 STURTEVANT suspected that the presence of a heterozygous inversion in one chromosome pair increases crossing over in a non-homologous pair of chromosomes. The effect was defini...

The powders of Ba(Ce1-xZrx)0.90Y0.1O3-δ (BCZY) with 0.2 ≤ x ≤ 0.6 have been prepared by a modified sol-gel method. Triethylenetetramine (TETA) was employed as chelating agent. Phase formation of calcined powders at 1100C and sintered pellets at 1400C of BCZY were examined by an X-ray diffractrometer (XRD). XRD results showed the calcined powder and sintered pellet formed a single perovskite pha...

The fibers extracted from the piassava palm tree, scientifically known as Attalea funifera, are among the stiffest lignocellulosic fibers being considered for polymer composite reinforcement. Characterization of piassava composites have been carried out for different polymeric matrices and mechanical tests. In this work the tensile properties of DGEBA/TETA epoxy matrix composites reinforced wit...