Window acoustic study for advanced turboprop aircraft

An acoustic analysis was performed to establish window designs for advanced turboprop powered aircraft. The window transmission loss requirements were based on A-weighted interior noise goals of 80 and 75 dBA. The analytical results showed that a triple pane window consisting of two glass outer panes and an inner pane of acrylic would provide the required transmission loss and meet the sidewall space limits. Two window test articles were fabricated for laboratory evaluation and verification of the predicted transmission loss. Procedures for performing laboratory tests are presented

Development and testing of cabin sidewall acoustic resonators for the reduction of cabin tone levels in propfan-powered aircraft

The use of Helmholtz resonators to increase the sidewall transmission loss (TL) in aircraft cabin sidewalls is evaluated. Development, construction, and test of an aircraft cabin acoustic enclosure, built in support of the Propfan Test Assessment (PTA) program, is described. Laboratory and flight test results are discussed. Resonators (448) were located between the enclosure trim panels and the fuselage shell. In addition, 152 resonators were placed between the enclosure and aircraft floors. The 600 resonators were each tuned to a propfan fundamental blade passage frequency (235 Hz). After flight testing on the PTA aircraft, noise reduction (NR) tests were performed with the enclosure in the Kelly Johnson Research and Development Center Acoustics Laboratory. Broadband and tonal excitations were used in the laboratory. Tonal excitation simulated the propfan flight test excitation. The resonators increase the NR of the cabin walls around the resonance frequency of the resonator array. Increases in NR of up to 11 dB were measured. The effects of flanking, sidewall absorption, cabin absorption, resonator loading of trim panels, and panel vibrations are presented. Resonator and sidewall panel design and test are discussed

Characterization of anticoagulant heparinoids by immunoprofiling

Heparinoids are used in the clinic as anticoagulants. A specific pentasaccharide in heparinoids activates antithrombin III, resulting in inactivation of factor Xa and–when additional saccharides are present–inactivation of factor IIa. Structural and functional analysis of the heterogeneous heparinoids generally requires advanced equipment, is time consuming, and needs (extensive) sample preparation. In this study, a novel and fast method for the characterization of heparinoids is introduced based on reactivity with nine unique anti-heparin antibodies. Eight heparinoids were biochemically analyzed by electrophoresis and their reactivity with domain-specific anti-heparin antibodies was established by ELISA. Each heparinoid displayed a distinct immunoprofile matching its structural characteristics. The immunoprofile could also be linked to biological characteristics, such as the anti-Xa/anti-IIa ratio, which was reflected by reactivity of the heparinoids with antibodies HS4C3 (indicative for 3-O-sulfates) and HS4E4 (indicative for domains allowing anti-factor IIa activity). In addition, the immunoprofile could be indicative for heparinoid-induced side-effects, such as heparin-induced thrombocytopenia, as illustrated by reactivity with antibody NS4F5, which defines a very high sulfated domain. In conclusion, immunoprofiling provides a novel, fast, and simple methodology for the characterization of heparinoids, and allows high-throughput screening of (new) heparinoids for defined structural and biological characteristics

SLC10A7 mutations cause a skeletal dysplasia with amelogenesis imperfecta mediated by GAG biosynthesis defects.

Skeletal dysplasia with multiple dislocations are severe disorders characterized by dislocations of large joints and short stature. The majority of them have been linked to pathogenic variants in genes encoding glycosyltransferases, sulfotransferases or epimerases required for glycosaminoglycan synthesis. Using exome sequencing, we identify homozygous mutations in SLC10A7 in six individuals with skeletal dysplasia with multiple dislocations and amelogenesis imperfecta. SLC10A7 encodes a 10-transmembrane-domain transporter located at the plasma membrane. Functional studies in vitro demonstrate that SLC10A7 mutations reduce SLC10A7 protein expression. We generate a Slc10a7-/- mouse model, which displays shortened long bones, growth plate disorganization and tooth enamel anomalies, recapitulating the human phenotype. Furthermore, we identify decreased heparan sulfate levels in Slc10a7-/- mouse cartilage and patient fibroblasts. Finally, we find an abnormal N-glycoprotein electrophoretic profile in patient blood samples. Together, our findings support the involvement of SLC10A7 in glycosaminoglycan synthesis and specifically in skeletal development