Wilson's disease is an orphan disease due to copper homeostasis dysfunction. Mutations of the ATP7B gene induces an impaired functioning of a Cu-ATPase, impaired Cu detoxification in the liver and copper overload in the body. Indeed, even though copper is an essential element, which is used as cofactor by many enzymes playing vital roles, it becomes toxic when in excess as it promotes cytotoxic...

Benthic fluxes of copper, copper complexing ligands and thiol compounds in the shallow waters of Venice Lagoon (Italy) were determined using benthic chambers and compared to porewater concentrations to confirm their origin. Benthic copper fluxes were small due to small concentration differences between the porewaters and the overlying water, and the equilibrium concentration was the same at bot...

Copper ions are essential at a proper level yet toxic when present in excess. To maintain a proper intracellular level, cells must be able to sense the changes in copper ion concentrations. The yeast transcription factor Mac1p plays a critical role in the transcriptional regulation of CTR1 and CTR3, both encoding high affinity copper ion transporters. Here we report that the Mac1p binding of th...

The voluntary withdrawal of chromated copper arsenate (CCA)-treated wood from most residential applications has increased the use of non-arsenical copper-based organic wood preservatives. Because the arsenic component of CCA controlled coppertolerant fungi, scientists have renewed interest in and concern about the decay capacity in the important copper-tolerant group of brown-rot fungi. We have...

Apparent digestibility coefficients (ADCs) of dry matter, crude protein, crude lipid, nitrogen-free extract, and energy in selected carbohydrate sources including wheat flour (WF), α-potato starch (PS), α-corn starch (CS), Na alginate (AL), dextrin (DEX), and carboxymethyl cellulose (CMC) were determined for olive flounder. The olive flounder averaging 150 ± 8.0 g were held in 300-L tanks at a ...

The apparent digestibility coefficients of three protein levels of reference diets (without fish meal) and test diets (with fish meal) were estimated for Labeo rohita. Three test diets (test diet-I, test diet-II and test diet-III) and three reference diets (reference diet-I, reference diet-II and reference diet-III) having 28, 30 and 32% protein levels were prepared. Chromic oxide was used as a...

The distribution of essential metal ions over subcellular compartments for use as cofactors requires control of membrane transporters. PAA2/HMA8 is a copper-transporting P1B -type ATPase in the thylakoid membrane, required for the maturation of plastocyanin. When copper is highly available to the plant this transporter is degraded, which implies the action of a protease. In order to identify th...

The health consequences of mild copper excess in humans are unknown. In a previous study, 2 mo of supplementation with up to 6 mg Cu/L in drinking water did not induce detectable changes. Here we assessed a copper supplement at the upper level of dietary recommendations for "healthy" adults. The study was a prospective controlled trial; participants (women and men, 18-50 y old), represented the...

Menkes disease is an X-linked disorder of copper metabolism. An overall copper deficiency reduces the activity of copper-dependent enzymes accounting for the clinical presentation of affected individuals. The Menkes gene product (MNK) is a P-type ATPase and is considered to be the main copper efflux protein in most cells. The protein is located primarily at the trans -Golgi network (TGN), but r...

As an essential trace element, copper can be toxic in mammalian cells when present in excess. Metallothioneins (MTs) are small, cysteine-rich proteins that avidly bind copper and thus play an important role in detoxification. Yeast CUP1 is a member of the MT gene family. The aim of this study was to determine whether yeast CUP1 could bind copper effectively and protect cells against copper stre...