The biliary excretion of copper was measured in eight patients with Wilson's disease (three untreated, with hepatic dysfunction) and 10 control subjects (three with hepatic dysfunction). The duodenum was perfused with an amino-acid solution containing a non-absorbed marker, 51CrC18, and juice was aspirated from the duodeno-jejunal junction. The mean concentration of copper in the duodenal aspir...

This study investigated the effects of accumulated copper, on digestive epithelium height and percentage area, and on oocyte numbers of the snail Helix aspersa, in a vineyard where copper oxychloride is sprayed. The ultimate aim was to determine the usefulness of these cellular responses as biomarkers. Results showed that snails collected 2 months after fungicide application, had a significantl...

Wilson's disease (WD) is a disorder of copper metabolism leading to copper accumulation in the liver and in extrahepatic organs, such as brain and cornea. We present a patient with liver disease who did not fulfil the biochemical criteria for WD. Mutational analysis was necessary to make the diagnosis and show a new mutation. Our case supports the use of mutation analysis in cases with unclear ...

The biliary excretion of copper was measured in eight patients with Wilson's disease (three untreated, with hepatic dysfunction) and 10 control subjects (three with hepatic dysfunction). The duodenum was perfused with an amino-acid solution containing a non-absorbed marker, (51)CrCl(3), and juice was aspirated from the duodeno-jejunal junction. The mean concentration of copper in the duodenal a...

INTRODUCTION Both primary biliary cirrhosis (PBC) and Wilson's disease (WD) can cause copper retention in the liver, which is an important factor for liver cellular damage. Copper chelation may preserve liver cell function. It is challenging to distinguish WD from copper accumulation in patients with PBC. There have been few case reports of PBC co-occurrence with WD. CASE PRESENTATION Here we...

The divalent metal ion transporter DMT1 is localized in the brush border membrane (BBM) of the upper small intestine and has been shown to be able to transport Mn2+, Fe2+, Co2+, Ni2+, and Cu2+. Belgrade rats have a glycine-to-arginine (G185R) mutation in DMT1, which affects its function. We investigated copper transport with BBM vesicles of Belgrade rats loaded with calcein, which exhibits fluo...

The accumulation of copper and zinc, the basic components of anti-fouling paints, was examined in cultured sea bass Dicentrarchus labrax and sea bream Sparus aurata. Samplings were carried out at 4 eastern Mediterranean fish farms. Two of the farms used nets treated with anti-fouling paints, and 2 used untreated nets. At each farm samples of sediment and fish tissue (muscle, liver and gills) we...

Copper is essential for aerobic life, but many aspects of its cellular uptake and distribution remain to be fully elucidated. A genome-wide screen for copper homeostasis genes in Drosophila melanogaster identified the SNARE gene Syntaxin 5 (Syx5) as playing an important role in copper regulation; flies heterozygous for a null mutation in Syx5 display increased tolerance to high dietary copper. ...

Copper is an essential trace element, but can become toxic when present in abundance. The severe effects of copper-metabolism imbalance are illustrated by the inherited disorders Wilson disease and Menkes disease. The Labrador retriever dog breed is a novel non-rodent model for copper-storage disorders carrying mutations in genes known to be involved in copper transport. Besides disease initiat...

Copper detoxification in the yeast Candida glabrata is carried out in large part by a family of metallothionein (MT) genes: a unique MT-I gene, a tandemly amplified MT-IIa gene, and a single unlinked MT-IIb gene. In response to elevated environmental copper levels, members of this MT gene family are transcriptionally activated by a copper-dependent, sequence-specific DNA-binding transcription f...