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Objective To summarize the clinical, muscle pathology and molecular biological features of late⁃onset glycogen storage disease type Ⅱ (GSDⅡ). Methods and Results Five patients with late⁃onset GSD Ⅱ diagnosed and treated in The Fifth Affiliated Hospital of Zhengzhou University from January 2013 to January 2020 were selected. The main clinical manifestations of 5 patients were weakness of raising the head, weakness of the proximal extremities, decreased muscle tone, fatigue intolerance, and dyspnea. The activity of acid α⁃glucosidase (GAA) was significantly reduced. HE staining of muscular tissues in 5 patients showed vacuole⁃like changes of different sizes, different numbers, and irregular shapes in most muscle fibers. Modified Gomori trichrome (MGT) staining showed a large number of blue and purple particles deposited in the vacuole. Periodic acid⁃Schiff (PAS) staining showed the glycogen content in the vacuoles were increased in 4 cases, and the glycogen content in the vacuoles were lost in one case. GAA gene testing showed that 9 mutations were detected in 5 patients, and 4 cases were compound heterozygous mutations, which were derived from father and mother respectively. The c.1320_1322delGAT (p.Met440del) was a deletion mutation, c.2331G＞C (p.Thr777Thr) was a synonymous mutation, c.2237G＞A (p.Trp746*) was a nonsense mutation, c.877G＞A (p.Gly293Arg) was a missense mutation, c.2238G＞C (p.Trp746Cys) was a missense mutation, c.784G＞A (p.Glu262Lys) was a missense mutation, c.2014C＞T (p.Arg672Trp) was a missense mutation, and c. 2332⁃2A＞G was a splicing mutation. One case was homozygous mutation of c. 1432G＞A (p. Gly478Arg), which originated from the mother. Among them, c. 2331G＞C, c. 1432G＞A and c. 2332⁃2A＞G were reported for the first time at home and abroad. Conclusions The clinical manifestations of late⁃onset GSD Ⅱ are weakness of proximal limbs and dyspnea. The activity of GAA in peripheral serum is decreased significantly. Muscle tissue pathology is characteristic. GAA gene mutations are mainly compound heterozygous mutation, c.2331G＞C, c.1432G＞A and c.2332⁃2A＞G are new mutations, which extended the mutation spectrum of GAA gene.

The type of organic solvents used in interfacial polymerization affects the surface property, free volume, and separation performance of the thin-film composite (TFC) polyamide membrane. In this study, TFC polyamide membrane was fabricated through interfacial polymerization between diethylenetriamine (DETA) and trimesoyl chloride (TMC). Four types of organic solvent were explored in the preparation of pervaporation membrane. These are tetralin, toluene, hexane, and isopentane. The solubility parameter distance between organic solvents and DETA follows in increasing order: tetralin (17.07 MPa1/2) < toluene (17.31 MPa1/2) < hexane (19.86 MPa1/2) < isopentane (20.43 MPa1/2). Same trend was also observed between the organic solvents and DETA. The larger the solubility parameter distance, the denser and thicker the polyamide. Consequently, field emission scanning electron microscope (FESEM) and positron annihilation spectroscopy (PAS) analysis revealed that TFCisopentane had the thickest polyamide layer. It also delivered the highest pervaporation efficiency (permeation flux = 860 ± 71 g m−2 h−1; water concentration in permeate = 99.2 ± 0.8 wt%; pervaporation separation index = 959,760) at dehydration of 90 wt% aqueous ethanol solution. Furthermore, TFCisopentane also exhibited a high separation efficiency in isopropanol and tert-butanol. Therefore, a suitable organic solvent in preparation of TFC membrane through interfacial polymerization enables high pervaporation efficiency.

Salinity is one of the most important abiotic stresses that adversely affects crop growth and productivity. A subtractive suppression hybridization (SSH) library were constructed from the roots of salt-sensitive Yulu cultivar melon seedlings under salt stress; 557 high-quality expressed sequence tags (ESTs) were randomly sequenced, with an average size of 428 bp, which assembled into 68 contigs and 315 singletons. Compared with our previous SSH library generated from the salt-tolerant Bingxuecui cultivar, the proportion of transcripts involved in metabolism, protein fate, cellular communication/signal transduction mechanisms, and cell rescue/defense were 4, 1.46, 0.94, and 0.4% higher, respectively, in the salt-tolerant cultivar than the in salt-sensitive cultivar. Quantitative real-time PCR analysis of eleven transcripts revealed temporal variations in their expression in the two cultivars under salt stress. One NAC gene (JZ477011) was heterologously expressed in yeast for functional characterization, and enhanced the sensitivity of yeast cells to high-salinity to salt stress and inhibited their growth. Information regards to their functions would aid in the understanding of response mechanisms to saline stress and in the development of molecular markers for selecting salt-tolerant melon cultivars.

A new lead complex, {[Pb(4-sb)(phen)](4,4′-bipy)0.5}n (1) (where 4-sb=4-sulfobenzoate dianion, phen=1,10-phenanthroline, 4,4′-bipy=4,4′-bipyridine), was synthesized and characterized by single-crystal X-ray, infrared, and thermogravimetric analyses and fluorescence studies. In this compound, the lead atom is hexacoordinated by two N atoms of one phen ligand and four O atoms from three 4-sb dianions. Each 4-sb serves as a bridging ligand, connecting adjacent PbII atoms into a centrosymmetric polymeric chain. Intermolecular π-π interactions and hydrogen bonds are observed in the complex, which stabilize the crystal packing and give rise to three-dimensional architecture.

In this work, CaO-rich briquette coke was prepared by carbonizing the cold pressing briquette composed of powdered char, CaO, and binders. The influences of binder type, binder content in briquette, the CaO/char ratio, and carbonization condition on compressive strength, resistivity, and porosity of the briquette were investigated. Subsequently, the optimum preparation conditions for producing CaO-rich briquetting coke were determined. The obtained results can promote development and optimization of in-situ production of calcium carbide and acetylene from CaO-rich briquetting coke. [ABSTRACT FROM AUTHOR]