Epoxidation of Propene by High-Throughput Screening Method Over Combinatorially Prepared Cu Catalysts Supported on High and Low Surface Area Silica

Gas phase epoxidation of propene using molecular oxygen was studied by use of a high-throughput testing technique. A large number of catalysts including promoted and un-promoted Cu were synthesized in a much faster combinatorial fashion using a sol-gel method. Metal catalysts supported on high and low surface area silica were tested and ranked in a high-throughput activity and selectivity testing apparatus at different experimental conditions such as reaction temperature and reactant gas ratio. The amount of Cu loading and the addition of alkali promoters such as K and Li resulted in different tendencies in consumption rate for both silica materials. The maximum PO production rate was obtained as 25.82 μmol/g/ cat./min (2.90 % conv. and 20.49 % selectivity) for 3 % Cu-2.25 % K catalyst supported on high surface area silica. There was no noticeable difference in structural and chemical properties of catalysts after modification with K when examined by XRD and TEM; however, the overall activation energy of un-modified catalysts (92 kJ/mol) decreased to 71 kJ/mol for K-modified catalyst. There was negligible difference between the activation energies calculated for PO production (75 vs. 77 kJ/mol)

Expanding the Mutational Spectrum of CRLF1 in Crisponi/CISS1 Syndrome

Crisponi syndrome (CS) and cold-induced sweating syndrome type 1 (CISS1) share clinical characteristics, such as dysmorphic features, muscle contractions, scoliosis, and cold-induced sweating, with CS patients showing a severe clinical course in infancy involving hyperthermia associated with death in most cases in the first years of life. To date, 24 distinct CRLF1 mutations have been found either in homozygosity or in compound heterozygosity in CS/CISS1 patients, with the highest prevalence in Sardinia, Turkey, and Spain. By reporting 11 novel CRLF1 mutations, here we expand the mutational spectrum of CRLF1 in the CS/CISS1 syndrome to a total of 35 variants and present an overview of the different molecular and clinical features of all of them. To catalog all the 35 mutations, we created a CRLF1 mutations database, based on the Leiden Open (source) Variation Database (LOVD) system (https://grenada.lumc.nl/LOVD2/mendelian_genes/variants). Overall, the available functional and clinical data support the fact that both syndromes actually represent manifestations of the same autosomal-recessive disorder caused by mutations in the CRLF1 gene. Therefore, we propose to rename the two overlapping entities with the broader term of Crisponi/CISS1 syndrome