8 research outputs found

    PLANT TESTS ON THE DECOMPOSITION OF NITROUS OXIDE OVER A HEATED RHODIUM CATALYST

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    Plant scale tests were conducted to determine the feasibility of decomposing the nitrous oxide in dissolver off gases with a heated rhodium catalyst. The test results from two fixed bed reactors operating on off gases containing between 14.2 and 19.8 vol.% nitrous oxide show that the nitrous oxide content can be reduced to less than 0.05% with catalyst bed outlet at 1300 to 1500 deg F and space velocities at 472 to 700 hr/sup -//sup 1/. Rate constants appear to be comparable to those reported in the literature. Suggestions are made for the design of a permanent installation. (auth

    International nuclear fuel cycle fact book. Revision 4

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    This Fact Book has been compiled in an effort to provide (1) an overview of worldwide nuclear power and fuel cycle programs and (2) current data concerning fuel cycle and waste management facilities, R and D programs, and key personnel in countries other than the United States. Additional information on each country's program is available in the International Source Book: Nuclear Fuel Cycle Research and Development, PNL-2478, Rev. 2. The Fact Book is organized as follows: (1) Overview section - summary tables which indicate national involvement in nuclear reactor, fuel cycle, and waste management development activities; (2) national summaries - a section for each country which summarizes nuclear policy, describes organizational relationships and provides addresses, names of key personnel, and facilities information; (3) international agencies - a section for each of the international agencies which has significant fuel cycle involvement; (4) energy supply and demand - summary tables, including nuclear power projections; (5) fuel cycle - summary tables; and (6) travel aids - international dialing instructions, international standard time chart, passport and visa requirements, and currency exchange rate

    HNRNPC haploinsufficiency affects alternative splicing of intellectual disability-associated genes and causes a neurodevelopmental disorder

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    Heterogeneous nuclear ribonucleoprotein C (HNRNPC) is an essential, ubiquitously abundant protein involved in mRNA processing. Genetic variants in other members of the HNRNP family have been associated with neurodevelopmental disorders. Here, we describe 13 individuals with global developmental delay, intellectual disability, behavioral abnormalities, and subtle facial dysmorphology with heterozygous HNRNPC germline variants. Five of them bear an identical in-frame deletion of nine amino acids in the extreme C terminus. To study the effect of this recurrent variant as well as HNRNPC haploinsufficiency, we used induced pluripotent stem cells (iPSCs) and fibroblasts obtained from affected individuals. While protein localization and oligomerization were unaffected by the recurrent C-terminal deletion variant, total HNRNPC levels were decreased. Previously, reduced HNRNPC levels have been associated with changes in alternative splicing. Therefore, we performed a meta-analysis on published RNA-seq datasets of three different cell lines to identify a ubiquitous HNRNPC-dependent signature of alternative spliced exons. The identified signature was not only confirmed in fibroblasts obtained from an affected individual but also showed a significant enrichment for genes associated with intellectual disability. Hence, we assessed the effect of decreased and increased levels of HNRNPC on neuronal arborization and neuronal migration and found that either condition affects neuronal function. Taken together, our data indicate that HNRNPC haploinsufficiency affects alternative splicing of multiple intellectual disability-associated genes and that the developing brain is sensitive to aberrant levels of HNRNPC. Hence, our data strongly support the inclusion of HNRNPC to the family of HNRNP-related neurodevelopmental disorders
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