A new Caenorhabditis elegans model to study copper toxicity in Wilson disease.

Wilson disease (WD) is caused by mutations in the ATP7B gene that encodes a copper (Cu) transporting ATPase whose trafficking from the Golgi to endo-lysosomal compartments drives sequestration of excess Cu and its further excretion from hepatocytes into the bile. Loss of ATP7B function leads to toxic Cu overload in the liver and subsequently in the brain, causing fatal hepatic and neurological abnormalities. The limitations of existing WD therapies call for the development of new therapeutic approaches, which require an amenable animal model system for screening and validation of drugs and molecular targets. To achieve this objective, we generated a mutant Caenorhabditis elegans strain with a substitution of a conserved histidine (H828Q) in the ATP7B ortholog cua-1 corresponding to the most common ATP7B variant (H1069Q) that causes WD. cua-1 mutant animals exhibited very poor resistance to Cu compared to the wild-type strain. This manifested in a strong delay in larval development, a shorter lifespan, impaired motility, oxidative stress pathway activation, and mitochondrial damage. In addition, morphological analysis revealed several neuronal abnormalities in cua-1 mutant animals exposed to Cu. Further investigation suggested that mutant CUA-1 is retained and degraded in the endoplasmic reticulum, similarly to human ATP7B-H1069Q. As a consequence, the mutant protein does not allow animals to counteract Cu toxicity. Notably, pharmacological correctors of ATP7B-H1069Q reduced Cu toxicity in cua-1 mutants indicating that similar pathogenic molecular pathways might be activated by the H/Q substitution and, therefore, targeted for rescue of ATP7B/CUA-1 function. Taken together, our findings suggest that the newly generated cua-1 mutant strain represents an excellent model for Cu toxicity studies in WD.

Valorization of Olive Mill Byproducts: Recovery of Biophenol Compounds and Application in Animal Feed.

This study aimed to recover the phenols from olive oil mill wastewater, a major pollutant of the oil industry, by using spray-drying technology to produce a new feed with a nutraceutical value for animal feed supplementation and to evaluate its effect on the productivity and nutritional quality of ewe milk. Forty-five Sarda ewes in late lactation (150 ± 2 d) and with homogeneous live weight (52 ± 1.5 kg) were randomly allotted into three groups and fed with three dietary treatments containing increasing levels of polyphenols: 0% (C), 0.1% (T0.1), and 0.2% (T0.2) of dry matter. No effect of the dietary treatments was found on the milk yield and composition. Interestingly, milk urea content (p < 0.0001) and somatic cell counts (p < 0.001) decreased as the level of polyphenols inclusion in the diet increased. The inclusion of phenols (0.2% of dry matter) in the diet of sheep was effective in increasing the vaccenic (C18:1 trans-11) and rumenic acid (C18: cis-9 trans-11) levels, which are beneficial for human health. Finally, the recovery of polyphenols via spray-drying technology and their incorporation into a new fortified feed can be a valid strategy for naturally improving the nutritional value of milk while valorizing an oil industry byproduct, reducing environmental impact, and promoting waste reuse that is in line with circular economy principles.

Local Small Ruminant Grazing in the Monti Foy Area (Italy): The Relationship Between Grassland Biodiversity Maintenance and Added-Value Dairy Products.

The literature indicates that grazing small ruminants, when adequately managed, contributes to grassland biodiversity maintenance. On the other hand, milk and cheese from grazing animals show higher nutritional and aromatic quality than those from stall-fed animals. The relationship between the two issues has rarely been addressed. This article provides information for a discussion of this relationship. First, two case studies are reported. Local breeds of small ruminants fed by grazing on pastures within the Special Area of Conservation "Monti Foy" in the Northwestern Basilicata region (Italy), with a stocking rate of 4.0 LU ha-1 year-1, showed the best effectiveness for the maintenance of grassland botanical biodiversity. Milk and cheese from pasture-fed goats showed higher contents of beneficial fatty acids, phenols, and vitamins A and E; higher degree of antioxidant protection; and richer volatile compound profiles, in particular for terpenes content. Finally, some recommendations for the management of grazing systems in similar mountain areas are offered, including a viable approach for land managers to preserve the grassland biodiversity of pastures and provide high-quality products that are valuable both for their nutritional quality and for their contribution to the economic sustainability of mountain communities.

Recent advances in dairy goat products.

Goat population world-wide is increasing, and the dairy goat sector is developing accordingly. Although the new technology applied to the goat industry is being introduced slowly because the weight of traditional subsector in the dairy sector, considerable advances have been made in the last decade. Present review focuses on the emerging topics in the dairy goat sector. Research and development of traditional and new dairy goat products are reviewed, including the new research in the use of goat milk in infant formula. The research in alternatives to brine, production of skimmed goat cheeses and the use of different modified atmosphere packaging are also addressed. Special attention is given to antibiotic residues and their determination in goat milk. Functional foods for human benefits are a trending topic. Health properties recently discovered in dairy goat products are included in the paper, with special attention to the antioxidant activity. The dual-purpose use of goats by humankind is affecting the way of how new technology is being incorporated in the dairy goat sector and will certainly affect the future development of dairy goat products.