Cs Ratio Research Articles

Rb3SiF7:Mn4+ and Rb2CsSiF7:Mn4+ Red-Emitting Phosphors with a Faster Decay Rate

A narrow-band red emission is a seminal issue for establishing the color-rendering index and color gamut of phosphor-converted white light emitting diode (pc-WLED) applications. In this regard, Mn4+-activated K2SiF6 phosphors (referred to as K216) have recently attracted a great deal of attention after their successful commercialization. As with K216 phosphors, Mn4+-activated K3SiF7 (referred to as K317) phosphors perform in a manner that is similar to that of K216 phosphors and have been introduced as a narrow-band red phosphor. Despite the acceptable performances of both versions of phosphors, slower decay remains a shortcoming for applications to high-powered LEDs. We introduced K317 derivatives by replacing K with Rb and Cs. As a result, Mn4+-activated (Rb,Cs)3SiF7 phosphors were synthesized with different Rb:Cs ratios and exhibited faster decay times by comparison with both the K216 and the K317 phosphors. The present study involved both structural and luminescent investigations along with density fu...

The ALMA-PILS survey: the sulphur connection between protostars and comets: IRAS 16293–2422 B and 67P/Churyumov–Gerasimenko

The evolutionary past of our Solar System can be pieced together by comparing analogous low-mass protostars with remnants of our Protosolar Nebula - comets. Sulphur-bearing molecules may be unique tracers of the joint evolution of the volatile and refractory components. ALMA Band 7 data from the large unbiased Protostellar Interferometric Line Survey (PILS) are used to search for S-bearing molecules in the outer disc-like structure, 60 au from IRAS 16293-2422 B, and are compared with data on 67P/C-G stemming from the ROSINA instrument aboard Rosetta. Species such as SO$_{2}$, SO, OCS, CS, H$_{2}$CS, H$_{2}$S and CH$_{3}$SH are detected via at least one of their isotopologues towards IRAS 16293-2422 B. The search reveals a first-time detection of OC$^{33}$S towards this source and a tentative first-time detection of C$^{36}$S towards a low-mass protostar. The data show that IRAS 16293-2422 B contains much more OCS than H$_{2}$S in comparison to 67P/C-G; meanwhile, the SO/SO$_{2}$ ratio is in close agreement between the two targets. IRAS 16293-2422 B has a CH$_{3}$SH/H$_{2}$CS ratio in range of that of our Solar System (differences by a factor of 0.7-5.3). It is suggested that the levels of UV radiation during the initial collapse of the systems may have varied and have potentially been higher for IRAS 16293-2422 B due to its binary nature; thereby, converting more H$_{2}$S into OCS. It remains to be conclusively tested if this also promotes the formation of S-bearing complex organics. Elevated UV levels of IRAS 16293-2422 B and a warmer birth cloud of our Solar System may jointly explain the variations between the two low-mass systems.

Analysis of Radionuclide Deposition Ratios from the Fukushima-Daiichi Incident.

This paper will examine deposition patterns of four radionuclides following the Fukushima-Daiichi accident. For nearly 13 d following the event, fission products were released into the environment through planned venting procedures and hydrogen explosions. To assist the government of Japan (GOJ) in the assessment of the releases, the National Nuclear Security Administration's Consequence Management Response Team (CMRT) deployed and took nearly 3 mo of measurements using airborne radiation sensors, fixed monitors, high purity germanium (HPGe) detectors, and health physics survey equipment. From the HPGe detector in-situ results gathered by the CMRT and GOJ teams, the depositions of Cs, Cs, Cs, and I were examined as a function of latitude and longitude. Deposition ratios were calculated to express how each radionuclide was deposited relative to Cs. In addition, the first 30 d of results were compared to the isotopic ratios listed in the Federal Radiological Monitoring and Assessment Center (FRMAC) Dose Assessment Manual Volume 2 Nuclear Power Plant default scenario. This was completed to analyze how the default FRMAC values compared with actual measurements. For Cs:Cs and Cs:Cs (1 wk after shutdown), the ratios were 0.969 ± 0.025 and 0.13 ± 0.007, respectively. These were significantly different from the FRMAC default values of 1.6 and 0.4, but they were of the same order of magnitude. Spatially, larger ratios with high uncertainties were recorded near Tokyo, over 200 km from the accident site. The I to Cs ratios, as expected, decayed exponentially over time but were significantly higher than FRMAC values. Six ratios were greater than 20 within 10 d after shutdown compared to the FRMAC default value of 9.9. In addition, the highest ratios were located less than 75 km to the southwest of the plant. Comparing all the isotopic ratios to the FRMAC manual illustrated differences between the default values and the actual field results. This is at least partly due to the fact that the FRMAC default values are based on an average between a pressurized water reactor and boiling water reactor release. These results of the comparison illustrate that the Assessment Manual default values should only be used when no other data are available.

Plasma-induced synthesis of chitosan-g-polyacrylamide and its flocculation performance for algae removal

ABSTRACTChitosan (CS)-g-polyacrylamide (PAM) is a highly efficient and environmentally friendly flocculant, which was synthesized through plasma-induced graft copolymerization of CS and acrylamide (AM). The effects of monomer concentration, AM:CS ratio, discharge power, discharge time, post-polymerization temperature, and post-polymerization time on the intrinsic viscosity, grafting ratio, and grafting efficiency of CS-g-PAM were investigated. The optimum conditions of graft copolymerization were as follows: 20% monomer concentration, 7:3 AM:CS ratio, 40 W discharge power, 90 s discharge time, 50°C post-polymerization temperature, and 24 h post-polymerization time. The structural characteristics of CS-g-PAM were characterized by Fourier-transform infrared spectroscopy, scanning electron microscopy, and X-ray diffraction. CS-g-PAM exhibited better flocculation efficiency than the commercially available PAM in both diatomite-simulated wastewater and low-turbidity algal water. The optimal turbidity removal efficiency for the diatomite-simulated wastewater was 99.9%, which was obtained with 6 mg L−1 of CS-g-PAM at pH 11.0 and 250 s−1 of velocity gradient. In low-turbidity algal water, the optimal removal efficiencies for chlorophyll-a, turbidity, and COD were 93.6%, 94.5%, and 98.2%, respectively.

Physical and Biological Properties of PCL/CS/nHA Composite Membranes for Guided Tissue Regeneration

With the aim to explore a barrier membrane with stable degradation rate and excellent biological properties for guided tissue regeneration (GTR), a series of PCL/ CS/nHA (100/0,95/5,90/10,85/15,80/20,w/w) composite membranes were composed via freeze drying. PCL/CS/nHA samples were divided into five groups by adjusting the PCL to CS ratio, and polyvinylpyrrolidone was used as porogen. The fabricated membranes were evaluated by mechanical properties, material degradation rate, morphological characterization and biological properties. Through these experiment, we can proved that for a PCL to CS ratio of 95/5 optimal conditions were as follows: porosity 31.25±2.35, water absorption 101.7, degradation 21.3%, tensile strength 19.2MPa, and elongation at break 218.6%. Moreover, in vitro cytologic research revealed that PCL/CS/nHA (95/5) composite membrane could efficiently increased the infiltration of periodontal ligament fibroblasts (PLFs). Finally, cell proliferation assay showed that PCL/CS/nHA (95/5) composite membrane could promote the differentiation and proliferation of PLFs. These results suggested that PCL/CS/nHA ((95/5) composite membrane could serve as a promising barrier membrane for guided tissue regeneration due to appropriate biodegradability, preferable mechanical properties and outstanding biocompatibility.

CO2 separation performance by chitosan/tetraethylenepentamine/poly(ether sulfone) composite membrane

ABSTRACTCO2 separation from CO2/N2 (20:80) gas mixture has been demonstrated by tetraethylenepentamine blended with chitosan (CS‐TEPA) membrane. Optimization of CS and TEPA weight ratio were carried out based on characterization details involving thermogravimetric analysis, Fourier transform infrared spectroscopy, X‐ray diffraction, atomic force microscope, and field emission scanning electron microscope. Effects of water flow rate, pressure, and temperature were concurrently studied on CS‐TEPA membranes through gas permeation. Almost twofold increase in CO2 permeance (24.7 GPU) was detected in CS blend with 30% (w/w) of TEPA (CS70) as compared to pure CS membrane (12.5 GPU). CS70 yielded CO2/N2 selectivity of 80 whereas CS demonstrated a maximum of 54 at 90 °C. The membrane also exhibited improved stability at temperatures less than 120 °C which was evident from TGA isotherm trace. The proposed composite membrane can be a promising candidate for flue gas separation. © 2017 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2017, 134, 45206.

Conditions for the formation of the chemical composition of the water of the Firuza MGU carbonated spring (Northern Pamirs)

We considered the geological conditions and chemical composition of the water of the Firuza MGU carbonated spring, which was discovered in 2015 in Northern Pamir. This paper reports on the detailed composition of the miсrocomponents of the water as determined by an Inductively Coupled Plasma Mass Spectrometry analysis (ICP-MS). A comparative review of the water composition and content of similar microcomponents in waters of carbonated springs in Kamchatka and the Russian Far East is given. On the basis of the B: Cl and K: Li: Rb: Cs ratios, which are close to the same ratios for chloride sodium hot springs in areas of active volcanism, it is concluded that the water of the spring has a deep origin. The temperature of the formation of water calculated by a Na–K geothermometer method is 147.3°C, suggesting the formation of the spring at a depth of approximately 5 km.

Simultaneous Bilateral Carotid Stenting for Symptomatic Bilateral High-Grade Carotid Stenosis: A Retrospective Clinical Investigation.

BackgroundThis retrospective clinical investigation aimed to evaluate the short-term effectiveness and safety of SBCAS for symptomatic bilateral high-grade CS.Material/MethodsFrom 2009 to 2014, 145 patients were recruited. Among them, 70 underwent SBCAS, and other 75 patients underwent SAMM and served as controls. The immediate postprocedural complications and postprocedural neurological evaluation, as well as restenosis at 6-month and 1-year follow-ups in the SBCAS group are reported. Additionally, baseline risk factors for ischemic stroke, adverse effects of drugs, and outcomes at 30-day, 6-month, and 1-year follow-ups were compared between the 2 groups.ResultsOur data did not reveal significant differences between the 2 groups in baseline risk factors for ischemic stroke. In the SBCAS group, both HPS (5.7%) and HD (40%) occurred, but they were not very severe, and no patients had postprocedural neurological deficit. Moreover, restenosis only occurred in 3 patients at 3 stent placement sites (4.3%) at 1-year follow-up. Adverse effects of drugs did not occur in SBCAS group, but adverse effects of Bayer aspirin and Lipitor occurred in 4 patients (5.4%) and 18 patients (24.3%), respectively, at 6-month follow-up in the control group. Furthermore, there were significant differences in outcomes between the 2 groups at 30-day, 6-month, and 1-year follow-ups, in that NIHSS, CS ratio, and incidence of endpoint events, as well as 1-year cumulative probability of endpoint events, were all lower in the SBCAS group than in the control group (p<0.05).ConclusionsCompared to SAMM, we found that SBCAS was more effective and safer for symptomatic bilateral high-grade CS.

Rutin-encapsulated chitosan nanoparticles targeted to the brain in the treatment of Cerebral Ischemia

ObjectiveRutin, a potent antioxidant, has been reported to reduce the risk of ischemic disease. Our study aims to prepare rutin-encapsulated-chitosan nanoparticles (RUT-CS-NPs) via ionic gelation method and determine its results, based on different parameters i.e. surface morphology characterization, in-vitro or ex-vivo release, dynamic light scattering and differential scanning calorimetry (DSC), for treating cerebral ischemia. MethodsUPLC-ESI-Q-TOF-MS/MS was used to evaluate the optimized RT-CS-NPs1 for brain-drug uptake as well as to follow-up the pharmacokinetics, bio-distrbution, brain-targeting efficiency and potential after intranasal administration (i.n.). Key findingsA particle size of <100nm for the formulation, significantly affected by drug:CS ratio, and entrapment efficiency and loading capacity of 84.98%±4.18% and 39.48%±3.16%, respectively were observed for RUT. Pharmacokinetics, bio-distribution, brain-targeting efficiency (1443.48±39.39%) and brain drug-targeting potential (93.00±5.69%) showed enhanced bioavailability for RUT in brain as compared to intravenous administration. In addition; improved neurobehavioral activity, histopathology and reduced infarction volume effects were observed in middle cerebral artery occlusion (MCAO) induced cerebral ischemic rats model after i.n. administration of RUT-CS-NPs. ConclusionA significant role of mucoadhesive-RT-CS-NPs1 as observed after high targeting potential and efficiency of the formulation prove; RUT-CS-NPs are more effectively accessed and target easily the brain.

Beyond body size: muscle biochemistry and body shape explain ontogenetic variation of anti-predatory behaviour in the lizard Salvator merianae.

Anti-predatory behaviour evolves under the strong action of natural selection because the success of individuals avoiding predation essentially defines their fitness. Choice of anti-predatory strategies is defined by prey characteristics as well as environmental temperature. An additional dimension often relegated in this multilevel equation is the ontogenetic component. In the tegu Salvator merianae, adults run away from predators at high temperatures but prefer fighting when it is cold, whereas juveniles exhibit the same flight strategy within a wide thermal range. Here, we integrate physiology and morphology to understand ontogenetic variation in the temperature-dependent shift of anti-predatory behaviour in these lizards. We compiled data for body shape and size, and quantified enzyme activity in hindlimb and head muscles, testing the hypothesis that morphophysiological models explain ontogenetic variation in behavioural associations. Our prediction is that juveniles exhibit body shape and muscle biochemistry that enhance flight strategies. We identified biochemical differences between muscles mainly in the LDH:CS ratio, whereby hindlimb muscles were more glycolytic than the jaw musculature. Juveniles, which often use evasive strategies to avoid predation, have more glycolytic hindlimb muscles and are much smaller when compared with adults 1-2 years old. Ontogenetic differences in body shape were identified but marginally contributed to behavioural variation between juvenile and adult tegus, and variation in anti-predatory behaviour in these lizards resides mainly in associations between body size and muscle biochemistry. Our results are discussed in the ecological context of predator avoidance by individuals differing in body size living at temperature-variable environments, where restrictions imposed by the cold could be compensated by specific phenotypes.

THE CONNECTING MOLECULAR RIDGE IN THE GALACTIC CENTER

We report new observations of multiple transitions of the CS molecular lines in the SgrA region of the Galactic center, at an angular resolution of 40" (=1.5 pc). The objective of this paper is to study the polar arc, which is a molecular ridge near the SgrA region, with apparent non-coplanar motions, and a velocity gradient perpendicular to the Galactic plane. With our high resolution dense-gas maps, we search for the base and the origin of the polar arc, which is expected to be embedded in the Galactic disk. We find that the polar arc is connected to a continuous structure from one of the disk ring/arm in both the spatial and velocity domains. This structure near SgrA* has high CS(J=4-3)/CS(J=2-1) ratios >1. That this structure has eluded detection in previous observations, is likely due to the combination of high excitation and low surface brightness temperature. We call this new structure the connecting ridge. We discuss the possible mechanism to form this structure and to lift the gas above the Galactic plane.

Preparation and properties of a novel environmentally friendly film from maleated poly(vinyl alcohol) grafted with chitosan in solution form: the effect of different production factors

A maleated poly(vinyl alcohol) (PVAM)-g-chitosan (CS) (PVAM-g-CS) film was prepared in solution form using potassium persulfate (K2S2O8) as an initiator. The influence of the PVAM:CS ratio, the reaction time and temperature and the concentration of K2S2O8 on the properties of the PVAM-g-CS were investigated. Ether linkage of the PVAM-g-CS was observed at 1154 and 1089 cm−1 by ATR-FTIR. The conditions for grafting CS to PVAM were developed and optimized. The highest degree of grafting was observed at a ratio of 9:1 PVAM:CS. The optimum grafting conditions were found to be 50 °C for a reaction time of 1 h, with a ratio of PVAM:CS of 9:1 and a concentration of 0.5 % K2S2O8. The maximum and minimum swelling ratios were 150 and 330 %, respectively. The tensile strength of the PVAM was enhanced after the incorporation of CS in the PVAM. The highest tensile strength of 24 MPa was found at a ratio of 5:5 PVAM:CS. The PVAM-g-CS shows a high efficiency of controlling capsaicin release in base–acid medium and was easily biodegradable in natural soil especially with high proportions of CS.

Biomarker responses correlate with antibody status in mucopolysaccharidosis type I patients on long-term enzyme replacement therapy

BackgroundAntibody formation can interfere with effects of enzyme replacement therapy (ERT) in lysosomal storage diseases. Biomarkers are used as surrogate marker for disease burden in MPS I, but large systematic studies evaluating the response of biomarkers to ERT are lacking. We, for the first time, investigated the response of a large panel of biomarkers to long term ERT in MPS I patients and correlate these responses with antibody formation and antibody mediated cellular uptake inhibition. MethodsA total of 428 blood and urine samples were collected during long-term ERT in 24 MPS I patients and an extensive set of biomarkers was analyzed, including heparan sulfate (HS) and dermatan sulfate (DS) derived disaccharides; total urinary GAGs (DMBu); urinary DS:CS ratio and serum heparin co-factor II thrombin levels (HCII-T). IgG antibody titers and the effect of antibodies on cellular uptake of the enzyme were determined for 23 patients. ResultsMedian follow-up was 2.3years. In blood, HS reached normal levels more frequently than DS (50% vs 12.5%, p=0.001), though normalization could take several years. DMBu normalized more rapidly than disaccharide levels in urine (p=0.02). Nineteen patients (83%) developed high antibody titers. Significant antibody-mediated inhibition of enzyme uptake was observed in 8 patients (35%), and this correlated strongly with a poorer biomarker response for HS and DS in blood and urine as well as for DMBu, DS:CS-ratio and HCII-T (all p<0.006). ConclusionsThis study shows that, despite a response of all studied biomarkers to initiation of ERT, some biomarkers were less responsive than others, suggesting residual disease activity. In addition, the correlation of cellular uptake inhibitory antibodies with a decreased biomarker response demonstrates a functional role of these antibodies which may have important clinical consequences.

Characterization of dielectrophoresis-aligned nanofibrous silk fibroin–chitosan scaffold and its interactions with endothelial cells for tissue engineering applications

Aligned three-dimensional nanofibrous silk fibroin–chitosan (eSFCS) scaffolds were fabricated using dielectrophoresis (DEP) by investigating the effects of alternating current frequency, the presence of ions, the SF:CS ratio and the post-DEP freezing temperature. Scaffolds were characterized with polarized light microscopy to analyze SF polymer chain alignment, atomic force microscopy (AFM) to measure the apparent elastic modulus, and scanning electron microscopy and AFM to analyze scaffold topography. The interaction of human umbilical vein endothelial cells (HUVECs) with eSFCS scaffolds was assessed using immunostaining to assess cell patterning and AFM to measure the apparent elastic modulus of the cells. The eSFCS (50:50) samples prepared at 10MHz with NaCl had the highest percentage of aligned area as compared to other conditions. As DEP frequency increased from 100kHz to 10MHz, fibril sizes decreased significantly. eSFCS (50:50) scaffolds fabricated at 10MHz in the presence of 5mM NaCl had a fibril size of 77.96±4.69nm and an apparent elastic modulus of 39.9±22.4kPa. HUVECs on eSFCS scaffolds formed aligned and branched capillary-like vascular structures. The elastic modulus of HUVEC cultured on eSFCS was 6.36±2.37kPa. DEP is a potential tool for fabrication of SFCS scaffolds with aligned nanofibrous structures that can guide vasculature in tissue engineering and repair.

Glutaraldehyde Cross linked Sodium Alginate / Chitosan pH Sensitive Microspheres for Controlled Release of Ibuprofen , Preparation and characterization

polymers of sodium alginate and chitosan (NaAlg/Cs) based pH-Sensitive interpenetrating network (IPN) microspheres (MPs) were prepared by emulsion- crosslinking technique using glutaraldehyde (GA) as a crosslinking agent. Ibuprofen, a non-steroidal anti-inflammatory drug (NSAID) was successfully encapsulated The biodegradable into the MPs by varying the ratio of % Cs, amount of GA and % drug loading. MPs with average particle sizes in the range of 293-372 µm were obtained. MPs were characterized by Fourier transform infrared spectroscopy (FTIR), Scanning electron microscopy (SEM) and Differential scanning calorimetry (DSC). In Vitro release studies were carried out in simulated gastric fluid of pH 1.2 for 2 h followed by simulated intestinal fluid of pH 7.4 at 37oC. In conclusion, the present study indicates that Ibuprofen was successfully encapsulated into the hydrophilic carbohydrate polymers blend MPs composed of NaAlg and Cs. The encapsulation efficiency up to 84% was obtained. The release of drug from the MPs was dependent upon the pH of medium, % Cs ratio, extent of GA and amount of drug loading. The n values ranged between 0.89 and 1.17, indicating that the drug release from the MPs shifted from anomalous to the super Case-II transport. FTIR confirmed the formations of IPN MPs. DSC have confirmed the amorphous dispersion of the drug in the polymer matrix.

Assessing the coefficient of the earth pressure at rest from shear wave velocity and electrical resistivity measurements

Determination of earth pressure coefficient K0 in natural soil deposits is a significantly important issue in geotechnical engineering. This paper evaluates the feasibility of predicting K0 values from measurements of vertical and horizontal shear wave velocities [Vs(VH), Vs(HH), and Vs(HV)], and vertical and horizontal electrical resistivities (SV, SH). A series of seismic piezocone tests (SCPTU), improved crosshole (CH) tests, and soil resistivity tests using a modified laboratory apparatus were carried out at two research sites in Jiangsu Province of China and results were used to develop two existing K0-shear wave velocity correlations for prediction of K0. The seismic and electrical resistivity test results showed that both the shear wave velocity ratios, Vs(HH)/Vs(HV) or Vs(HV)/Vs(VH), and the horizontal to vertical electrical resistivity ratio, SH/SV, may provide a good indicator of the inherent soil anisotropy at these sites. The existing Vs–K0 prediction framework largely requires determining Cs(HV)/Cs(HH) ratio and a simple procedure for evaluating this ratio based on the measured horizontal and vertical electrical resistivities. The K0 values predicted by shear wave velocity at both sites are also compared with values determined by Jaky's formula and Mayne & Kulhwy's formula. In normally consolidated (NC) soils, the K0 values predicted from these methods showed good agreement. However, for over-consolidated (OC) soils at shallow depths, certain variations between different methods are noted. Further investigations of the effects of overconsolidation ratio on K0 predictions will lead to the correction factors for better prediction of K0 for OC soils. This paper has confirmed that reasonable predictions of K0 for natural soil deposits can be made using the proposed methodology.

Colorless antibacterial cotton fabrics based on silver nanoparticles and chitosan complexes

PurposeThe purpose of this paper is to fabricate colorless cotton fabrics with good antibacterial activity and durability.Design/methodology/approachChitosan (CS) based silver nanoparticles (AgNPs) were formed in CS solutions as the antibacterial agent. The reducing agent was sodium borohyride. The concentrations of the CS solutions ranged from 0.1 to 1 percent (w/v). Cotton fabrics were impregnated by these CS/AgNPs solutions.FindingsAll of these fabrics exhibited superior antibacterial activities. The antibacterial activity still showed great efficiency even after 81 home launderings. Moreover, the results of color change and whiteness indicated that the cotton fabrics treated by CS/AgNPs complex with higher CS concentration had less color change compared with other samples.Practical implicationsFabrics treated by this method could reduce the brown color brought by AgNPs. The paper also suggests that cotton fabrics treated by AgNPs formed in a relatively higher CS concentration not only had good antibacterial activity but also were colorless.Originality/valueThe influence of CS ratio in CS/AgNPs complexes on the antibacterial activity and color of cotton fabrics was studied, which has been rarely reported in previous papers. The fabrics prepared by this method are promising candidates for a wide range of general applications.

Serum HCII-T and urinary DS:CS ratio are both predictive biomarkers of treatment outcome in patients with MPS I, II and VI

Serum HCII-T and urinary DS:CS ratio are both predictive biomarkers of treatment outcome in patients with MPS I, II and VI

Heparin cofactor II‐thrombin complex and dermatan sulphate:chondroitin sulphate ratio are biomarkers of short‐ and long‐term treatment effects in mucopolysaccharide diseases

Early detection of mucopolysaccharidosis (MPS) is an important factor in treatment success; therefore, good disease biomarkers are vital. We evaluate heparin cofactor II-thrombin complex (HCII-T) as a biomarker in serum and dried blood spots (DBS) of MPS patients. Serum HCII-T and urine dermatan sulphate:chondroitin sulphate (DS:CS) ratio are also compared longitudinally against clinical outcomes in MPSI, II and VI patients following treatment. Samples were collected from MPS patients at the Royal Manchester Children’s Hospital. DS:CS ratio was obtained by measuring the area density of spots from 2D electrophoresis of urinary glycosaminoglycans. Serum and DBS HCII-T was measured by sandwich ELISA. Serum HCII-T is elevated approximately 25-fold in MPS diseases that store DS, clearly distinguishing untreated MPSI, II and VI patients from unaffected age-matched controls. Serum HCII-T is also elevated in MPSIII, which leads to storage of heparan sulphate, with an increase of approximately 4-fold over unaffected age-matched controls. Urine DS:CS ratio and serum HCII-T decrease in response to treatment of MPSI, II and VI patients. HCII-T appears to respond rapidly to perturbations in treatment, whilst DS:CS ratio responds more slowly. HCII-T is a suitable biomarker for MPSI, II and VI, and it may also be informative for MPS diseases storing HS alone, such as MPSIII, although the elevation observed is smaller. In treated MPS patients, HCII-T and DS:CS ratio appear to measure short-term and long-term treatment outcomes, respectively. The potential value of HCII-T measurement in DBS for newborn screening of MPS diseases warrants further investigation.

Investigation of substitution effects and the phase transition in type-I clathrates Rb xCs 8–xSn 44□ 2 (1.3≤ x≤2.1) using single-crystal X-ray diffraction, Raman spectroscopy, heat capacity and electrical resistivity measurements

The substitution of cations in Rb x Cs 8– x Sn 44□ 2(1.3≤ x≤2.1) is reported. The compounds crystallize at room temperature in the space group la 3 ¯ d adopting the type-I clathrate 2×2×2 superstructure with partly ordered framework vacancies (□), whereas at higher temperatures they transform to the primitive, more disordered modification (space group Pm 3 ¯ n ). The guest atom distributions in the Sn cages on the Rb: Cs ratios is studied by means of single-crystal X-ray diffraction for Rb 2.1(1)Cs 5.8(1)Sn 44 at T=293 K ( 1), Rb 1.42(8)Cs 6.58(8)Sn 44 at T=293 K ( 2a), Rb 1.46(5)Cs 6.54(5)Sn 44 at T=373 K ( 2b) and Rb 1.32(8)Cs 6.68(8)Sn 44 at T=293 K ( 3). The structural order–disorder phase transition influences the electrical resistivity. The hysteresis observed for the electrical resistivity in combination with the symmetric shape of the specific heat anomaly suggests that the transformation is of first-order type and is characterized by an entropy change of about 2.5 J mol –1 K –1. The Raman spectrum for the low-temperature modification of 2 is also reported.