Mutations in CCNO result in congenital mucociliary clearance disorder with reduced generation of multiple motile cilia
NATURE GENETICS
Authors: Wallmeier, Julia; Al-Mutairi, Dalal A.; Chen, Chun-Ting; Loges, Niki Tomas; Pennekamp, Petra; Menchen, Tabea; Ma, Lina; Shamseldin, Hanan E.; Olbrich, Heike; Dougherty, Gerard W.; Werner, Claudius; Alsabah, Basel H.; Koehler, Gabriele; Jaspers, Martine; Boon, Mieke; Griese, Matthias; Schmitt-Grohe, Sabina; Zimmermann, Theodor; Koerner-Rettberg, Cordula; Horak, Elisabeth; Kintner, Chris; Alkuraya, Fowzan S.; Omran, Heymut
Abstract
Using a whole-exome sequencing strategy, we identified recessive CCNO (encoding cyclin O) mutations in 16 individuals suffering from chronic destructive lung disease due to insufficient airway clearance. Respiratory epithelial cells showed a marked reduction in the number of multiple motile cilia (MMC) covering the cell surface. The few residual cilia that correctly expressed axonemal motor proteins were motile and did not exhibit obvious beating defects. Careful subcellular analyses as well as in vitro ciliogenesis experiments in CCNO-mutant cells showed defective mother centriole generation and placement. Morpholino-based knockdown of the Xenopus ortholog of CCNO also resulted in reduced MMC and centriole numbers in embryonic epidermal cells. CCNO is expressed in the apical cytoplasm of multiciliated cells and acts downstream of multicilin, which governs the generation of multiciliated, cells. To our knowledge, CCNO is the first reported gene linking an inherited human disease to reduced MMC generation due to a defect in centriole amplification and migration.
A life-cycle inventory for the production of oleochemical raw materials
TENSIDE SURFACTANTS DETERGENTS
Authors: Hirsinger, F; Schick, KP; Stalmans, M
Abstract
An overview is given of the resource and energy requirements and tire environmental emissions arising from the production of the oleochemical raw materials crude palm oil (CPO), crude palm kernel oil (CPKO), crude coconut oil (CCNO) and tallow (Ta). The total energy requirements range from 47 GJ/1000 kg (CPO) to 65 GJ/1000 kg (CCNO), die energy of material resource ranges from 66% (Ta) to 96% (CCNO) of the total energy. Fossil energy sources comprise only 4% (CCNO) to 21% (Ta) of the total energy input. For CPO, process related emissions contribute most of the particulates, sulphur oxides, methane, sulphur and non-fossil CO2 whereas NOx, CO and fossil CO2 comprise most of the fuel related emissions. For CPKO, particulates, methane, hydrocarbons, SOx, CO and fossil CO2 emissions are process related. For CCNO, process emissions contribute most of the particulates and non-fossil CO2 emissions whereas fuel related emissions are mostly related to NOx, hydrocarbons, SOx and fossil CO2. For tallow, most of the process related emissions are methane, amonia, non-fossil CO2 and N2O whereas fuel-related emissions contribute to most of the NOx, hydrocarbons, SOx, CO and fossil CO2. Fur all the oleochemical raw materials, waterborne emissions are primarily process related For tallow, many of the process related waterborne emissions are associated with phosphate fertilizer production whereas, for the vegetable oils, most of the BOD and COD emissions are associated with the clarification processes and wastewater treatment during the milling processes. For all raw materials, process related solid waste accounts for about 90% of the total solid waste. In the case of CPO and CPKO, the listed process-related solid waste is actually ''fuel-related'' because of self sufficient energy production by the burning of fibres and shells. Most of the process related solid waste for coconut oil or for crude coconut oil production comes from coconut harvesting and husking, since coconut husks are not being utilized as compost in the plantation for 1% of all coconuts in the Philippines. For tallow, most of the process related solid waste is due to clay ''slime'' resulting from the processing of phosphate fertilizer from phosphate rock.