Wilson's disease (WD) is a rare hereditary disease caused by a deficiency of the ATF7B transporter. The accumulation of copper can cause damage to organs and cells, mainly the liver. Copper exposure can modulate cytokine synthesis through molecular and cellular signaling pathways, including the nuclear transcription factor NF-kB pathway. NF-kB is the main regulator of inflammation and cell death, acts as a central link between liver damage, fibrosis and hepatocellular carcinoma. An excess of NF-kB-dependent cytokine response stimulates inflammatory reactions, but excessive inhibition of NF-kB can negatively affect the viability of hepatocytes. Method of flow cytometry with visualization — Amnis ImageStreamX allows to evaluate the activity of NF-kB (% of activated cells in cell populations). The aim: to evaluate the activity of NF-kB in lymphocyte populations in children with WD disease. Immunophenotyping of lymphocytes and assessment of the level of translocation of NF-kB were performed in 52 children with WD and in 25 children of comparison group. The mass concentration of copper in daily urine was determined by atomic absorption method using the AAnalyst 800 spectrometer. In children with WD, the content of cells with NF-kB translocation varied from 5 to 90% depending on the lymphocyte population; the highest level was detected in B cells — 57.5 (37-68) %. A significant difference in distributions of the number of cells with NF-kB translocation between WD and healthy children was shown (F-criterion, p < 0.01). In most cases, children with WD are characterized by a decrease in the activity of NF-kB in populations of B cells (in 43% of cases), T helper cells (48%), T cytotoxic (44%) and Th17 lymphocytes (41%). In children with WD, the concentration of copper varied from 9.7 to 2582 mcg/day, Me = 616 (210-1173). A direct relationship was obtained between the copper content in urine and the level of translocation of NF-kB in B lymphocytes, r = 0.34, p = 0.016. The activity of the NF-kB correlates with biochemical markers of the severity of liver damage (ALT, AST, GGT) and with copper content in urine. The study of the NF-kB signaling pathway seems promising for a better understanding of the pathogenetic mechanisms of the formation of inflammation and liver fibrosis in children with WD.

Nonferrous and rare metals are mined mainly in deposits in which the ore bodies have complex structures and the composition of the ore is very far from constant. The ore bodies have irregular shapes with numerous branchings and pinch and swell are frequently observed. The "pattern" of the reserves is disturbed both within the ore body itself and in the veins or zones. The ore composition is characterized by the presence of several types and grades of varying commercial value, the processing of which requires a variety of beneficiation schemes.Open-cut mining of deposits of nonferrous and rare metals is performed by general or selective methods. The general method is widely employed owing to its simpllnity and therefore the relatively low cost per ton of ore. However, this method is not always effective: cutting and removal are accompanied by high losses and impoverishment of the ore, sometimes reaching 10 and 300, respectively. The simultaneous mining of different types and grades of ore leads to increased losses during beneficiation.Removal of the mineral with greater efficiency and lesser contamination of the ore is obtained by selective mining, particularly if systems of complex selective removal are employed [1]. We can then employ separate processing of the various ore grades in the beneficiation plant, thus increasing the extractable value per ton of ore by a factor of 1.5-2.5.In separate removal, the drilling and blasting work and the loading operations undergo maximum variations because the operational characteristics and procedures change. The mining equipment widely used in all ore quarries is employed for drilling and loading. Selective removal of the ore by excavators is performed by various scooping procedures and with various locations of the excavator at the face. The way in which a particular scooping procedure is performed depends on the skill of the operator rather than on the excavator's design characteristics. Even if the work is handled carefully, losses and impoverishment of the ore are inevitable because, as the bucket scoops up the broken rock, it "wanders" up the working face and captures material outside the ore body or the individual ore (rock) inclusions. Therefore the drilling and loading equipment used in most quarries does not permit selective working. The mining of highgrade ore and the reduction of losses of metal therefore demand new engineering solutions in this field.The most essential step in the working of complex faces is to change the scooping procedure; this requires the development of new loaders: the forces developed on the teeth of the bucket must enable it to "bite" at any level.The Mining Institute of the Siberian Section of the Academy of Sciences of the USSR and Gipronlkel' are developing loaders with "activated" buckets for underground working [2]. Similar machines are being designed abroad [3]. For example, the GSR-11 has a bucket with five picks, actuated by a compressed-air or electrohydraullc drive, with impact energy 4.5 kgm and impact frequency 940 per min. T...

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