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Leber's hereditary optic neuropathy (LHON) is a maternally inherited eye disease. X-linked nuclear modifiers were proposed to modify the phenotypic manifestation of LHON-associated mitochondrial DNA (mtDNA) mutations. By whole-exome sequencing, we identified the X-linked LHON modifier (c.157C>T, p.Arg53Trp) in PRICKLE3 encoding a mitochondrial protein linked to biogenesis of ATPase in 3 Chinese families. All affected individuals carried both ND4 11778G>A and p.Arg53Trp mutations, while subjects bearing only a single mutation exhibited normal vision. The cells carrying the p.Arg53Trp mutation exhibited defective assembly, stability, and function of ATP synthase, verified by PRICKLE3-knockdown cells. Coimmunoprecipitation indicated the direct interaction of PRICKLE3 with ATP synthase via ATP8. Strikingly, cells bearing both p.Arg53Trp and m.11778G>A mutations displayed greater mitochondrial dysfunction than those carrying only a single mutation. This finding indicated that the p.Arg53Trp mutation acted in synergy with the m.11778G>A mutation and deteriorated mitochondrial dysfunctions necessary for the expression of LHON. Furthermore, we demonstrated that Prickle3-deficient mice exhibited pronounced ATPase deficiencies. Prickle3-knockout mice recapitulated LHON phenotypes with retinal deficiencies, including degeneration of retinal ganglion cells and abnormal vasculature. Our findings provided new insights into the pathophysiology of LHON that were manifested by interaction between mtDNA mutations and X-linked nuclear modifiers.

China is rich in lithium resource, among which lithium ore in the Salt Lake has huge reserves, but the development and utilization technology has yet to be developed, and the main target of development is hard-rock lithium ore at present. Hard-rock lithium ore is mainly pegmatitic and concentrated in Xinjiang and Sichuan. The Mesozoic is the most important metallogenic period for pegmatite-type lithium deposits in China. A relatively stable tectonic environment after an orogeny is favored by the formation of pegmatite-type lithium deposits. After a decade of exploration practice and theoretical research, the types of lithium resource in China have shown a great variety. The brine-type lithium resource has expanded from surface brine to both shallow brine and deep brine, and the hard-rock lithium resources from single granitic pegmatite-type to altered granite-type, cryptoexplosion breccia tube-type and sedimentary-type. The metallogenic age has extended from the Meso-Cenozoic to the Paleozoic and other epochs. The metallogenic zones has increased from 12 to 16, and a number of new mineralized areas have been discovered in the Jiajika and Ke’eryin areas in western Sichuan and the Dahongliutan and Shaligou areas in Xinjiang . A new resource pattern of lithium is being formed. Moreover, Prospecting methods and exploration techniques have also developed from single surface prospecting and mapping to an integration of new techniques and methods, e.g., remote sensing to determine the prospective area, geological surveying to determine the type, geochemical prospecting to determine the mineral, geophysical prospecting to determine the location of drilling, drilling to determine the reserves, as well as biological prospecting, deep penetration of the deep exploration. In view of the rigid demand for lithium resources due to the rapid development of strategic emerging industries, it is suggested to strengthen a) the investigation, research, development and utilization of new types of lithium resources with lepidolite as the main industrial mineral and sedimentary-type lithium resources with Li-bearing clay as the main lithium resources; b) the research and prospecting of the Paleozoic and even Precambrian lithium deposits; c) the exploration of new lithium mineralization belts such as Altyn Tagh, Himalaya, Gangdise and western slope of Great Hinggan Mountains; d) the research on new mechanisms of dynamic management of lithium resources under market economy; e) the advanced research and resource reserve on lithium isotope as the raw material of controllable nuclear fusion, for leading the development of high-end mining industry.

BACKGROUND With the development of high-precision negative thermal ionization mass spectrometry (N-TIMS) technology, Re-Os isotopic dating of carbonaceous mudstone is more and more widely used in the determination of sedimentary age. The controversy over the diagenetic and metallogenic ages of the Dabaoshan copper deposit in Guangdong Province has always been a key restricting the study of the genetic mechanism. OBJECTIVES To constrain the diagenetic and metallogenic ages of the Dabaoshan copper deposit. METHODS Re-Os isotope ratios were determined by high precision N-TIMS after dissolving carbonaceous mudstone powder by reverse aqua regia in the Carius tube. Zircon U-Pb age was determined by laser ablation-inductively coupled plasma-mass spectrometry (LA-ICP-MS). RESULTS (1) The contents of Re and Os ranged from 0.976×10-9 to 2.997×10-9 and 0.067×10-9 to 0.115×10-9, respectively. The 187Re/188Os ratio varied from 78.236 to 154.799, and the 187Os/188Os ratio varied from 1.642 to 1.885. The Re-Os isotopic isochron age of black carbonaceous mudstone was 195±28Ma (n=6, MSWD=17). (2) The 206Pb/238U age ranged from 2631 to 236Ma, including two Mesozoic zircons, accounting for 2.50% of the total zircons, 19 Paleozoic zircons, accounting for 23.8% of the total zircons, 58 proterozoic zircons, accounting for 73% of the total zircons, 1 Archean zircon, accounting for 1% of the total zircons. The results of detrital zircon U-Pb age were broad and developed multiple peak periods, among which the youngest source magmatic zircon was formed in the Indosinian period. CONCLUSIONS The diagenetic and metallogenic age of the Dabaoshan copper deposit in Guangdong Province was constrained to the early Yanshanian. The distribution characteristics of detrital zircon U-Pb age further indicate that the Lower Jurassic Jinji Formation was formed at the stable continental margin, and its detrital material comes mainly from the ancient continental basement.

Product architecture represented the arrangements and combinations of physical components. It might promote innovation of product diversity, component sharing, and meet customer's personalized needs. Open design might support customers to participate in product development, and open product architecture provided platform strategy for personalized product development. To further study the theory and applications of product architecture, the concept and effect of product architecture were introduced, as well as the characteristics, association and difference between single product architecture, product family architecture and open product architecture were expounded. The evolution processes of product architecture and their internal motivation were analyzed from the aspects of architecture topology, architecture development strategy, architecture organization and management. The concept, connotation, function and mechanism of product architecture were reviewed. The dependence of customers personalized needs, product architecture and enterprise's open innovation strategy were discussed, and then open design strategies of product architecture were explored. [ABSTRACT FROM AUTHOR]

An additive flame retardant was prepared from DOPO-HQ. Fourier transform infrared spectroscopy (FT-IR) was used to analyze its chemical structure. It was added to the experimental formula to prepare EP composite material, and the limiting oxygen index experiment and thermogravimetric analysis experiment were carried out on the material, and the scanning electron microscope experiment was carried out on the residual carbon after combustion. The results show that the modified epoxy resin has a limiting oxygen index of 29.5%, and there is no droplet during combustion; thermogravimetric analysis experiments show that the temperature at which the thermal weight loss is 5%wt decreases, but the residual carbon rate increases. Scanning electron microscopy (SEM) test results show that carbon layer formed by the combustion products of epoxy resin is denser..

The Jiajika rare metal deposit contains the largest area of granitic pegmatite-type rare metal deposits in China. The X03 vein is an immense rare metal deposit dominated by lithium, which was found in the deposit in recent years. The contact metamorphic belt of tourmalinization and petrochemistry is widely developed in its wall rocks, and the altered rocks formed contain Li and other rare metal mineralization. In this paper, the tourmaline found in the different rocks of the Jiajika X03 vein is divided into four types: two-mica quartz schist (Tur-Ⅰ), tourmaline hornfels (Tur-Ⅱ), tourmaline-bearing granite pegmatite (Tur-Ⅲ) and spodumene-bearing granite pegmatite (Tur-Ⅳ); their in situ major element, trace element and boron isotope data are systematically studied. The results show that all tourmalines in the Jiajika X03 vein deposit belong to the alkali group, and are schorl–Oxy/Fluor–schorl, dravite–Hydroxy-dravite and foitite–Oxy foitite solid solutions, among which Tur-Ⅰ are dravite, Tur-Ⅱ are foitite of hydrothermal origin and Tur-Ⅲ and Tur-Ⅳ are schorl of magmatic origin. The boron isotope values show that the boron involved the formation process of tourmaline mainly originates from the Majingzi S-type granite, and the boron isotope variations in tourmaline are controlled by melt fluid and Rayleigh fractionation. Moreover, there is a clear correlation between the B isotope value of tourmaline and the Li, Mn, Zn, Mg, and V contents, showing that these contents in tourmaline are good indicators of the mineralization type of pegmatite. [ABSTRACT FROM AUTHOR]