Functional and Molecular Properties of DYT-SGCE Myoclonus-Dystonia Patient-Derived Striatal Medium Spiny Neurons

Myoclonus-dystonia (DYT-SGCE, formerly DYT11) is characterized by alcohol-sensitive, myoclonic-like appearance of fast dystonic movements. It is caused by mutations in the SGCE gene encoding ε-sarcoglycan leading to a dysfunction of this transmembrane protein, alterations in the cerebello-thalamic pathway and impaired striatal plasticity. To elucidate underlying pathogenic mechanisms, we investigated induced pluripotent stem cell (iPSC)-derived striatal medium spiny neurons (MSNs) from two myoclonus-dystonia patients carrying a heterozygous mutation in the SGCE gene (c.298T>G and c.304C>T with protein changes W100G and R102X) in comparison to two matched healthy control lines. Calcium imaging showed significantly elevated basal intracellular Ca²⁺ content and lower frequency of spontaneous Ca²⁺ signals in SGCE MSNs. Blocking of voltage-gated Ca²⁺ channels by verapamil was less efficient in suppressing KCl-induced Ca²⁺ peaks of SGCE MSNs. Ca²⁺ amplitudes upon glycine and acetylcholine applications were increased in SGCE MSNs, but not after GABA or glutamate applications. Expression of voltage-gated Ca²⁺ channels and most ionotropic receptor subunits was not altered. SGCE MSNs showed significantly reduced GABAergic synaptic density. Whole-cell patch-clamp recordings displayed elevated amplitudes of miniature postsynaptic currents and action potentials in SGCE MSNs. Our data contribute to a better understanding of the pathophysiology and the development of novel therapeutic strategies for myoclonus-dystonia.     

Impact of dietary n−3 FA deficiency on rat bone tissue FA composition

The effect of dietary n−3 FA deficiency on bone tissue FA composition was evaluated in growing rats. Two mixtures combining hydrogenated coconut oil with safflower oil served as the n−3-deficient dietary treatments and provided two levels of linoleic acid (LA). The n−3 treatments were formulated with added α-linolenic acid (LNA) from flaxseed oil (diet LNA) or LNA plus DHA, and both were balanced for LA. This study showed that bone is sensitive to changes in dietary n−3 FA and that DHA is more effective than LNA in maintaining DHA levels in these tissues.     

妊娠相关血浆蛋白A的分离及纯化

采用人胎盘血为原料,利用Sephadex G-200,DEAE-Sephadex A-50层析,Sepharose 4B反相免疫亲和层析技术,获得妊娠相关血浆蛋白A(PAPP-A)纯品,经8.0％聚丙烯酰胺凝胶电泳、免疫电泳、双向免疫扩散证明,所提取的PAPP-A纯品为单一成分。免疫家兔获得抗血清,其效价在1:16以上。     

Cuticular hydrocarbons ofReticulitermes virginicus (Banks) and their role as potential species- and caste-recognition cues

The cuticular hydrocarbon components of four castes ofReticulitermes virginicus (Banks) have been identified and quantitated. Components identified includen-alkanes; 2-, 3-, 11-, 13-, and 15-methyl-alkanes; 11,15-dimethylalkanes, (Z)-9-alkenes; (Z,Z)-7,9-dienes; and (E/Z)-6,9-dienes ranging in carbon number from C₂₁ to C₄₀. All caste forms ofR.virginicus contained the same components, but showed caste-specific proportions. Comparison of these hydrocarbons with those of the sympatric termiteR. flavipes (Kollar) suggest that cuticular hydrocarbons might serve as species- and caste-recognition cues. A bioassay was developed to test this species-recognition hypothesis, with the experimental results supporting the hypothesis.Isoptera: Rhinotermitidae.     

Cuticular lipids of the booklouse,Liposcelis bostrychophila: hydrocarbons,aldehydes, fatty acids,and fatty acid amides

The booklouse, Liposcelis bostrychophila, is an increasingly common pest of stored food products worldwide. We report here the cuticular lipid composition of this pest (the first report of the hydrocarbons of any member of the Order Psocoptera and the first report of fatty acid amides as cuticular components for any insect). No unsaturated hydrocarbons were present. A homologous series of n-alkanes (C₂₁–C₃₄), monomethyl alkanes (3-, 4-, 5-, 7-, 9-, 11-, 12-, 13- and 15-methyl-) with a carbon chain range of C₂₈–C₄₂, and dimethyl alkanes (3, 7-; 9, 13-; 11, 15-; 13, 17-; 9, 21-; 11, 19-; and 13, 21-); with a carbon number range of C₃₁–C₄₃ were identified. The relative abundances of these hydrocarbons were low, comprising approximately 0.0125% of total biomass. The amides were a homologous series (C₁₆–C₂₂ in chain length), with the major amide being stearoyl amide. In addition to the amides, free fatty acids (C_16:1, C_16:0, C_18:2, C_18:1, and C_18:0 in chain length) and three straight chain aldehydes (C₁₅, C₁₆, and C_17:1 in chain length) also occurred as cuticular components. These findings are discussed in terms of the chemical and physiological ecology of this species.     

Desulfurization of commercial fuels by π-complexation: Monolayer CuCl/γ-Al2O3

Monolayer CuCl/γ-Al₂O₃ sorbent was studied for desulfurization of a commercial jet fuel (364.3 ppmw S) and a commercial diesel (140 ppmw S). The sorbent was prepared by means of spontaneous monolayer dispersion methods. Deep desulfurization (sulfur levels of <1 ppmw) was accomplished with this sorbent using a fixed-bed adsorber. The CuCl/γ-Al₂O₃ sorbent was capable of removing 6.4 and 11.2 mg of sulfur per gram for jet fuel at breakthrough (at <1 ppmw S) and saturation, respectively. The same sorbent was capable of removing 0.94 and 1.8 mg of sulfur per gram for BP diesel at breakthrough and saturation, respectively. The difference in sulfur capacities for jet fuel and diesel was apparently caused by the difference in concentrations of strongly binding compounds, such as nitrogen heterocycles, heavy (polynuclear) aromatics and fuel additives. In comparison with CuCl/γ-Al₂O₃, Cu(I)Y zeolite has higher sulfur capacities but is less stable and can be easily oxidized to Cu(II)Y by fuel additives (such as oxygenates) and moisture and consequently loses π-complexation ability. However, all these cuprous π-complexation sorbents selectively adsorb thiophenic compounds over aromatics and olefins (as predicted by the high separation factors), which resulted in the observed desulfurization capability. A feasibility study is shown for efficient regeneration of CuCl/γ-Al₂O₃ using ultrasound at ambient temperature. Possible problems associated with desulfurization using π-complexation sorbents for commercial fuels are discussed.     

Epoxy fatty acid ester plasticizers. Preparation and properties

Summary The preparation of epoxy fatty acid ester plasticizers has been reviewed. These plasticizers can be prepared by epoxidation reactions, utilizing a preformed peracid or anin-situ technique. A newin-situ epoxidation process, utilizing hydrogen peroxide and acetic acid in the presence of strong acid catalyst, has been described. Excellent conversions to epoxide have been achieved with a series of monounsaturated and polyunsaturated esters. Epoxy fatty acid esters are excellent plasticizers for vinyl resins. Their performance in a series of additional epoxy plasticizers of vegetable and marine origin is described. Novel epoxy plasticizers based upon mixed acetylated mono- and di-glyceride have been prepared and are found to have excellent plasticizer performance. Presented at the 28th fall meeting, American Oil Chemists’ Society, October 1954, Minneapolis, Minn.     

Raman spectra of Graphon carbon black

The Raman spectrum of Graphon carbon black has been recorded using rotating cell techniques. Angular dependence of scattering at 1360, 1580 and 2700 cm^?1 are reported and these data suggest that the 1360 cm^?1 line is associated with non-planar microstructure distortions. The excitation frequency dependence of the intensity ratio of the bands at 1360 (D) and 1580 cm^?1 (G) is interpreted in terms of resonance (vibronic) interaction. This dependence is primarily the result of an increase in the intensity of the 1360 cm ^?1 line. The disorder-associated line (D) exhibits a significant excitation-dependent shift from 1378 cm^i?1 (457.9 nm Ar⁺) to 1330 cm^?1 (647.1 nm Kr⁺). The “graphite” (G) line position is less sensitive to changes in excitation frequency. The spectral features are discussed in terms of factor group, C_6v⁴, and layer site symmetry, C_3v. Also the possible role of localized alkene-like structure in zones of structural distortion is considered.     

High-Thermal-Conductivity Aluminum Nitride Ceramics: The Effect of Thermodynamic, Kinetic, and Microstructural Factors

Improvement in the thermal conductivity of aluminum nitride (AlN) can be realized by additives that have a high thermodynamic affinity toward alumina (Al₂O₃), as is clearly demonstrated in the aluminum nitride-yttria (AlN-Y₂O₃) system. A wide variety of lanthanide dopants are compared at equimolar lanthanide oxide:alumina (Ln₂O₃: Al₂O₃, where Ln is a lanthanide element) ratios, with samaria (Sm₂O₃) and lutetia (Lu₂O₃) being the dopants that give the highest- and lowest-thermal-conductivity AlN composites, respectively. The choice of the sintering aid and the dopant level is much more important than the microstructure that evolves during sintering. A contiguous AlN phase provides rapid heat conduction paths, even at short sintering times. AlN contiguity decreases slightly as the annealing times increase in the range of 1–1000 min at 1850°C. However, a substantial increase in thermal conductivity results, because of purification of AlN grains by dissolution-reprecipitation and bulk diffusion. Removal of grain-boundary phases, with a concurrent increase in AlN contiguity, occurs at high annealing temperatures or at long times and is a natural consequence of high dihedral angles (poor wetting) in liquidphase-sintered AlN ceramics.     

Chlorination of polystyrene

Chlorination of a low molecular weight polystyrene in the α position was studied by following changes in the infrared spectrum, glass transition temperature, and molecular weights of the polymer. The logarithm of the absorbance ratio at 2920 and 1500 cm^?1 as a function of mole per cent chlorination was linear. The glass transition temperatures, determined by use of a differential scanning calorimeter (DSC), were found to obey an equation developed by Dyvik for copolymers. The molecular weight of the chlorinated polystyrene decreased as a function of chlorination.