Usefulness of Trichoscopy over Hair Light Microscopy in Menkes Disease

Menkes disease (MD) is a rare X-linked recessive neurodegenerative disorder caused by mutations in the <i>ATP7A</i> gene, with a high mortality rate within the first 3 years of life. It typically affects males and is characterized by impaired copper distribution and malfunction of several copper-dependent enzymes. Patients develop progressive muscle hypotonia associated with neurological damage and hair shaft dysplasia – particularly pili torti. Pili torti is usually very subtle in the first 3 months of life and gradually increases during the first year. Light microscopy examination in search for pili torti requires the observation of more than 50 hair shafts. In contrast, trichoscopy with a hand-held dermatoscope allows to easily identify the hair shaft defect. We report a case of a Hispanic male infant with MD in whom we show that trichoscopy is superior to hair light microscopy in revealing pili torti.

Counteracting the Negative Effects of Copper Limitations Through the Biostimulatory Action of a Tropical Plant Extract in Grapevine Under Pedo-Climatic Constraints

In southern Mediterranean areas, vineyards are facing the combination of increasing air temperature, drought and frequency of extreme events (e.g., heat waves) due to climate change. Since most of the berry growth and ripening phases occur during the aridity period, such environmental constraints are responsible for limitations in yield and berry quality. Within this scenario, to achieve vineyard sustainability, renewed approaches in vineyard management have been proposed and the use of plant biostimulants seems a prominent and environmental friendly practice. The aim of this study was to test four combinations of a tropical plant extract and conventional chemicals for disease control on morpho-anatomical, physiological, biochemical and berry quality in Vitis vinifera L. subsp. vinifera “Aglianico.” In particular, we aimed to evaluate the possibility to counteract the negative effects of the reductions in copper distribution, by applying the tropical plant extract enriched with: micronutrients, enzymes involved in the activation of natural defense, aminoacids, and vitamins. The halved dose of Cu in combination with the tropical plant extract allowed maintaining a reduced vegetative vigor. In the second year of treatment, the addition of the plant extract significantly improved leaf gas exchanges and photochemistry as well as the synthesis of photosynthetic pigments. At berry level, the plant extract induced an increase in phenolics accompanied by a decrease in soluble sugars. The overall results showed that the expected differences in growth performance and productivity in vines are linked to different eco-physiological and structural properties induced by the various treatments. The tropical plant extract also primed plant defenses at the leaf and fruit levels, mainly due to modifications of some structural and biochemical traits, respectively.

Relationship between Down-Regulation of Copper-Related Genes and Decreased Ferroportin Protein Level in the Duodenum of Iron-Deficient Piglets

In mammals, 2 × 1012 red blood cells (RBCs) are produced every day in the bone marrow to ensure a constant supply of iron to maintain effective erythropoiesis. Impaired iron absorption in the duodenum and inefficient iron reutilization from senescent RBCs by macrophages contribute to the development of anemia. Ferroportin (Fpn), the only known cellular iron exporter, as well as hephaestin (Heph) and ceruloplasmin, two copper-dependent ferroxidases involved in the above-mentioned processes, are key elements of the interaction between copper and iron metabolisms. Crosslinks between these metals have been known for many years, but metabolic effects of one on the other have not been elucidated to date. Neonatal iron deficiency anemia in piglets provides an interesting model for studying this interplay. In duodenal enterocytes of young anemic piglets, we identified iron deposits and demonstrated increased expression of ferritin with a concomitant decline in both Fpn and Heph expression. We postulated that the underlying mechanism involves changes in copper distribution within enterocytes as a result of decreased expression of the copper transporter—Atp7b. Obtained results strongly suggest that regulation of iron absorption within enterocytes is based on the interaction between proteins of copper and iron metabolisms and outcompetes systemic regulation.

Copper distribution in organs and tissues of albino rats under oral administration of nanocomposite of copper oxide encapsulated in a polymeric matrix of arabinogalactan

Introduction. Based on arabinogalactan, a complex of hybrid nanobiocomposites has been created, which carry a balanced amount of macro- and microelements necessary for the body. A feature of the action of nanoparticles on the body is their ability to easily penetrate all organs and tissues. The aim of the work is to study the effect of copper arabinogalactan on the content of copper in the main tissues and organs of rats. Material and methods. The content of copper in organs and tissues of rats after a 10-day intragastric administration of a solution of copper arabinogalactan in a dose of 500 μg per kg of weight was studied by the atomic absorption method. Results. A high level of copper (above 2 μg/g) was found in wool, kidneys, liver, and heart. The brain and testes contain from 1 to 2 μg/g of copper. In the tissues of the stomach, small and large intestines, thymus, pancreas, blood, eyes, spleen, lungs, and skeletal muscles - less than 1 μg/g. Conclusion. The study of the distribution of copper in the organs of rats after oral administration of the nanocomposite of copper oxide with arabinogalactan demonstrated the main target organs for the accumulation of the element to be the kidneys, liver, brain, and stomach. The different character of the accumulation of copper in the tissues of the body of the rats received arabinogalactan of copper in the form of a nanocomposite, may indicate the selective ability of tissues and organs to accumulate this element. For further work on the possible use of the drug for medicinal purposes, it is necessary to take into account the peculiarities of biodistribution and bioaccumulation in various organs.

Fabrication Study of Cu-C-Ni for EDM Electrode by a Sintering Technique

The aim of this research was to study the feasibility for applications related to powder metallurgy in EDM electrode fabrication by combination of the percentage Cu-C-Ni element. The experiment was performed by comparing preliminary distribution results of particle size before and after the grinding of metal powders. The work-piece was pressed at a pressure of 200 Psi using a uniaxial press machine. The pressed green compact work-piece was then baked in a furnace. The gas inside the furnace that was used to control the temperature during the cold press procedure had a mixture gas ratio between argon and hydrogen of 95:5 (common grade) at 1030 oC and a soaking time of one hour. The results were examined by comparing the electric resistivity property, apparent density, bulk density as well as the porosity percentage inside the work piece material. The results revealed that the optimum combination of percentage Cu-C-Ni element was Cu92-C3-Ni5 (%wt), leading to the satisfactory Copper distribution in most of the structure. The size of sub sieve powder after grinding was found to be 22-31 micron with an electric resistivity of 1.45829E-05 k-ohm*cm. The minimum porosity percentage was found to be 2.19 %. Therefore, the element properties were found to be suitable for using as an electrode in EDM work when compared to that of the electrode prototype (EDM C3).