Gains of glycosylation comprise an unexpectedly large group of pathogenic mutations

Mutations involving gains of glycosylation have been considered rare, and the pathogenic role of the new carbohydrate chains has never been formally established. We identified three children with mendelian susceptibility to mycobacterial disease who were homozygous with respect to a missense mutation in IFNGR2 creating a new N-glycosylation site in the IFNgammaR2 chain. The resulting additional carbohydrate moiety was both necessary and sufficient to abolish the cellular response to IFNgamma. We then searched the Human Gene Mutation Database for potential gain-of-N-glycosylation missense mutations; of 10,047 mutations in 577 genes encoding proteins trafficked through the secretory pathway, we identified 142 candidate mutations ( approximately 1.4%) in 77 genes ( approximately 13.3%). Six mutant proteins bore new N-linked carbohydrate moieties. Thus, an unexpectedly high proportion of mutations that cause human genetic disease might lead to the creation of new N-glycosylation sites. Their pathogenic effects may be a direct consequence of the addition of N-linked carbohydrate.     

Electroless copper-phosphorus coatings with the addition of silicon carbide (SiC) particles

Cu-P-silicon carbide (SiC) composite coatings were deposited by means of electroless plating. The effects of pH values, temperature, and different concentrations of sodium hypophosphite (NaH₂PO₂·H₂O), nickel sulfate (NiSO₄·6H₂O), sodium citrate (C₆H₅Na₃O₇·2H₂O) and SiC on the deposition rate and coating compositions were evaluated, and the bath formulation for Cu-P-SiC composite coatings was optimised. The coating compositions were determined using energy-dispersive X-ray analysis (EDX). The corresponding optimal operating parameters for depositing Cu-P-SiC are as follows: pH 9; temperature, 90℃; NaH₂PO₂·H₂O concentration, 125 g/L; NiSO₄·6H₂O concentration, 3.125 g/L; SiC concentration, 5 g/L; and C₆H₅Na₃O₇·2H₂O concentration, 50 g/L. The surface morphology of the coatings analysed by scanning electron microscopy (SEM) shows that Cu particles are uniformly distributed. The hardness and wear resistance of Cu-P composite coatings are improved with the addition of SiC particles and increase with the increase of SiC content.     

Recrystallization and mechanical properties of WE43 magnesium alloy processed via cyclic expansion extrusion

In this study, cyclic expansion extrusion (CEE), as a relatively new severe plastic deformation (SPD) process, is applied to a rare earth (RE) containing Mg alloy WE43. The effects of the processing temperature and the number of passes are also investigated. The results showed that dynamic recrystallization (DRX) occurred after CEE processing at 400℃, and a bimodal structure with ultrafine DRXed grains surrounded the unrecrystallized grains. However, the DRX at 330℃ was retarded because of the existence of RE elements. The tensile tests showed that a simultaneous increase in the strength and the ductility of WE43 is obtained after CEE processing at 400℃ via two passes. Furthermore, the highest ultimate tensile strength of 440 MPa was achieved after the second pass of CEE at 330℃, and the highest ductility of 21% was attained after the second pass of CEE at 400℃. The microhardness measurements showed that the hardness increased from HV 80 to HV 114 and HV 98 after two passes of CEE processing at 330 and 400℃, respectively. In conclusion, increasing the processing passes could increase the mechanical properties and the volume fraction of the recrystallized grains. Moreover, increasing the temperature reduced the strength and the microhardness even if the elongation increased.     

Microstructure and mechanical properties of Al/Cu/Mg laminated composite sheets produced by the ARB proces

In the present study, an Al/Cu/Mg multi-layered composite was produced by accumulative roll bonding (ARB) through seven passes, and its microstructure and mechanical properties were evaluated. The microstructure investigations show that plastic instability occurred in both the copper and magnesium reinforcements in the primary sandwich. In addition, a composite with a perfectly uniform distribution of copper and magnesium reinforcing layers was produced during the last pass. By increasing the number of ARB cycles, the microhardness of the layers including aluminum, copper, and magnesium was significantly increased. The ultimate tensile strength of the sandwich was enhanced continually and reached a maximum value of 355.5 MPa. This strength value was about 3.2, 2, and 2.1 times higher than the initial strength values for the aluminum, copper, and magnesium sheets, respectively. Investigation of tensile fracture surfaces during the ARB process indicated that the fracture mechanism changed to shear ductile at the seventh pass.