Lafora disease (LD) and adult polyglucosan body disease (APBD) are glycogen storage diseases characterized by a pathogenic buildup of insoluble glycogen. Mechanisms causing glycogen insolubility are ...poorly understood. Here, in two mouse models of LD (Epm2a−/− and Epm2b−/−) and one of APBD (Gbe1ys/ys), the separation of soluble and insoluble muscle glycogen is described, enabling separate analysis of each fraction. Total glycogen is increased in LD and APBD mice, which, together with abnormal chain length and molecule size distributions, is largely if not fully attributed to insoluble glycogen. Soluble glycogen consists of molecules with distinct chain length distributions and differential corresponding solubility, providing a mechanistic link between soluble and insoluble glycogen in vivo. Phosphorylation states differ across glycogen fractions and mouse models, demonstrating that hyperphosphorylation is not a basic feature of insoluble glycogen. Lastly, model-specific variances in protein and activity levels of key glycogen synthesis enzymes suggest uninvestigated regulatory mechanisms.
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•Soluble glycogen content and structure: WT-like in Epm2a−/−, Epm2b−/−, Gbe1ys/ys mice•Glycogen hyperphosphorylation is not a general feature of insoluble glycogen•Abnormal chain length and molecule size distributions are limited to insoluble glycogen•Wild-type glycogen molecules vary in average chain length and precipitation propensity
EPM2A, EPM2B, or GBE1 deficiency causes insoluble glycogen accumulation and neurodegenerative diseases. Sullivan et al. show that these defects do not impair the construction of WT-like soluble glycogen. Demonstrating varying chain length distributions and correlating precipitation propensity among WT-glycogen molecules, a mechanistic explanation emerges for the structural characteristics of insoluble glycogen.
The dual-function glass dielectric resonator antenna (DRA) that simultaneously serves as a light cover is investigated for the first time. This paper is the first part, which demonstrates the idea ...using omnidirectional hollow rectangular glass DRAs. Both linearly polarized (LP) and circularly polarized (CP) designs are given. The glass DRA can generate CP fields by adding metallic patches onto its side walls, with a 3-dB axial ratio bandwidth of ~ 7%. To obtain an entirely light-transmissible CP glass DRA, conducting indium tin oxide (ITO) patches are used in place of the metallic patches. For each of the LP and CP designs, powered light emitting diodes (LEDs) are placed inside the hollow region of the glass DRA to serve as the light source. It is shown that practically the lighting and antenna parts do not interfere with each other. The reflection coefficient, axial ratio (CP designs only), radiation patterns, and antenna gains of the proposed LP and CP antennas are studied, and good agreement between measured and simulated results is observed.
To determine the characteristics of bloodstream infections (BSIs) and to evaluate the impact of BSIs on mortality in severe burn patients.
A retrospective observational study was conducted in 20 ...tertiary hospitals. A total of 185 patients who experienced a massive dust explosion in eastern China were included.
After exclusion, 177 patients were analysed. The median total body surface area (TBSA) burned was 95% (interquartile range 85%–98%). Inhalation injuries occurred in 97.2%. The overall 90-day mortality was 35% (62/177). During the study period, 120 (67.8%) patients developed 253 episodes of BSI with 323 unique causative pathogens. Sixty-six episodes were polymicrobial infections. Catheter-related BSIs (CRBSIs) accounted for 41.5% of the episodes. Acinetobacter baumannii (19.5%), Klebsiella pneumoniae (13.9%) and Candida (12.7%) were the most common organisms. Antimicrobial resistance was found in 63.5% of the isolates, particularly in Gram-negative bacteria. Patients who developed BSIs had a greater illness severity at admission to the intensive care unit, and worse outcomes. After adjusting for demographics, severity of illness and treatment characteristics in a multivariate logistic model, there was a trend toward BSI increasing the risk of 90-day mortality (adjusted OR 3.4; 95% CI 0.9–12.9; p=0.069). In subgroup analyses, CRBSIs (adjusted OR 5.7; 95% CI 1.3–24.9; p=0.021 versus no BSI) and polymicrobial BSIs (adjusted OR 6.1; 95% CI 1.3–28.1; p=0.020 versus no BSI) had greater risk of 90-day mortality.
A strikingly high rate of BSIs was observed in severe burn patients. Gram-negative organisms and fungi were the leading causes. CRBSIs and polymicrobial BSIs were associated with high mortality.
Cadmium oxide (CdO) thin films were prepared by pulsed laser deposition technique. Their structure, surface morphology, optical and electrical properties have been investigated. With a decrease in ...the laser energy density, the average grain size of the CdO film can be adjusted from 108 to 25 nm. High-resolution TEM observation showed that more crystalline defects like lattice distortion, dislocation and amorphous structure existed in the small grained (25 nm) CdO film, and X-ray photoelectron spectroscopy analysis confirmed that the film had more oxygen vacancies. The electrical and optical properties of the films significantly depended on the grain size. With the grain size decreasing to 25 nm, the optical band gap energy of the CdO film increased obviously from 2.82 to 3.33 eV. This change in the nature of material from semimetal to a wide band gap semiconductor, combining with its higher optical transmission (92 %) in visible light region, higher carrier concentration (1.25 × 10
21
cm
−3
) and lower electrical resistivity (2.8 × 10
−4
cm
−3
), makes the nano-grained CdO film very useful in optoelectronic applications.
Studies of Helicobacter pylori from the West have linked production of vacuolating cytotoxin and a particular signal sequence (s1a) allele of the underlying vacA gene to peptic ulcer disease (PUD). ...Among Chinese H. pylori, most isolates from both PUD and gastritis patients were toxigenic (35/46 and 29/35, respectively). Polymerase chain reaction and DNA sequencing showed that 95 of 96 isolates carried vacA s1a alleles. In the mid-region, 78 of 96 isolates carried m2; 14 were m1-like but only 87% identical (DNA-level) to classical m1 and were designated m1b; the other 4 were unusual hybrids (m1b-type proximal, m2-type distal). Isolates with m1b and m1b-m2 alleles produced higher levels of vacuolating activity than did isolates with m2 alleles (P < .01). There was no association between any vacA allele and disease. These results suggest that the composition of H. pylori gene pools varies geographically and that other as-yet-unknown polymorphic H. pylori genes are important in PUD.
A supersonic molecular beam injection (SMBI) system has been developed as a new fueling method for the J-TEXT tokamak. According to experimental needs, the system can provide various supersonic gas ...jets with the pressure range 0.2–2.0 MPa and pulse duration 0.2–10 ms. In this paper, the structure of the system is described in detail, including injectors, controller and gas line. Wave forms of the controller output show a fast response of the system. The evolution of pressure curve in the vacuum chamber indicates the system works well. Test results also show that the particle number of SMBI is linear proportional to the product of gas pressure and pulse duration. For different pressure of 0.5, 1.0 and 1.66 MPa, the beam velocities are estimated about 0.9, 1.2 and 1.4 km/s, respectively. Besides, fuelling experiments with the SMBI system are performed. Significant density increase demonstrates the fuelling abilities of both LSMBI and HSMBI.
Summary
We investigated the role of insulin‐like growth factor‐1 (IGF‐1) in spontaneously hypertensive rats with erectile dysfunction. Firstly, we evaluated intracavernous pressure. The ...bioavailability of IGF‐1 at both mRNA and protein levels were measured by quantitative real‐time PCR and Western blot respectively. Then, cavernous cyclic guanosine monophosphate concentrations were detected by enzyme‐linked immunosorbent assay. The cavernosal pressure was significantly decreased in the hypertensive and the propranolol treatment groups compared to the normal control group (P < 0.01). Cavernous IGF‐1 bioavailability and the concentrations of cavernous cyclic guanosine monophosphate were both significantly decreased in the hypertensive and the propranolol treatment groups compared to the normal control group (P < 0.01). This study suggests that an obvious decrease in cavernous IGF‐1 levels might play an important role in spontaneously hypertensive rats with erectile dysfunction.
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