From Biomass to Fuel Blendstocks via Catalytic Fast Pyrolysis and Hydrotreating: An Evaluation of Carbon Efficiency and Fuel Properties for Three Pathways.

Biomass was upgraded to fuel blendstocks via catalytic fast pyrolysis (CFP) followed by hydrotreating using three approaches: ex situ CFP with a zeolite catalyst (HZSM-5), ex situ CFP with a hydrodeoxygenation catalyst (Pt/TiO2) and cofed hydrogen, and in situ CFP with a low-cost mixed metal oxide catalyst (red mud). Each approach was evaluated using a common pine feedstock and the same hydrotreating procedure. The oxygen contents in the CFP oils ranged from 17 to 28 wt % on a dry basis, and the carbon efficiencies for the CFP processes were in the range of 28-38%. The residual oxygen was reduced to <1 wt % during hydrotreating, which was operated for 104-140 h for each CFP oil without plugging issues. The hydrotreating carbon efficiencies were 81-93%. The CFP pathway with the hydrodeoxygenation catalyst gave the highest overall carbon efficiency from biomass to fuel blendstocks (34%) but, at the same time, also the highest cumulative hydrogen consumption during CFP and hydrotreating. The zeolite pathway produced the largest fraction boiling in the gasoline range and the highest estimated octane number due to the high aromatic content in that CFP oil. The in situ red mud pathway produced the largest fraction of diesel-range products with the highest derived cetane number. However, advances in the CFP and hydrotreating process are required to improve the fuel blendstock properties for all pathways.

PUF60-related developmental disorder: A case series and phenotypic analysis of 10 additional patients with monoallelic PUF60 variants.

PUF60-related developmental disorder (also referred to as Verheij syndrome), resulting from haploinsufficiency of PUF60, is associated with multiple congenital anomalies affecting a wide range of body systems. These anomalies include ophthalmic coloboma, and congenital anomalies of the heart, kidney, and musculoskeletal system. Behavioral and intellectual difficulties are also observed. While less common than other features associated with PUF60-related developmental disorder, for instance hearing impairment and short stature, identification of specific anomalies such as ophthalmic coloboma can aid with diagnostic identification given the limited spectrum of genes linked with this feature. We describe 10 patients with PUF60 gene variants, bringing the total number reported in the literature, to varying levels of details, to 56 patients. Patients were recruited both via locally based exome sequencing from international sites and from the DDD study in the United Kingdom. Eight of the variants reported were novel PUF60 variants. The addition of a further patient with a reported c449-457del variant to the existing literature highlights this as a recurrent variant. One variant was inherited from an affected parent. This is the first example in the literature of an inherited variant resulting in PUF60-related developmental disorder. Two patients (20%) were reported to have a renal anomaly consistent with 22% of cases in previously reported literature. Two patients received specialist endocrine treatment. More commonly observed were clinical features such as: cardiac anomalies (40%), ocular abnormalities (70%), intellectual disability (60%), and skeletal abnormalities (80%). Facial features did not demonstrate a recognizable gestalt. Of note, but remaining of unclear causality, we describe a single pediatric patient with pineoblastoma. We recommend that stature and pubertal progress should be monitored in PUF60-related developmental disorder with a low threshold for endocrine investigations as hormone therapy may be indicated. Our study reports an inherited case with PUF60-related developmental disorder which has important genetic counseling implications for families.

Environmentally Friendly New Catalyst Using Waste Alkaline Solution from Aluminum Production for the Synthesis of Biodiesel in Aqueous Medium.

Red mud (RM) is composed of a waste alkaline solution (pH = 13.3) obtained from the production of alumina. It contains high concentrations of hematite (Fe2O3), goethite (FeOOH), gibbsite [Al(OH)3], a boehmite (AlOOH), anatase (Tetragonal-TiO2), rutile (Ditetragonal dipyramidal-TiO2), hydrogarnets [Ca3Al2(SiO4)3-x(OH)4x], quartz (SiO2), and perovskite (CaTiO3). It was shown to be an excellent catalytic mixture for biodiesel production. To demonstrate the value of RM, an environmentally friendly process of transesterification in aqueous medium using waste cooking oil (WCO), MeOH, and waste alkaline solution (WAS) obtained from aluminum production was proposed. Triglycerides of WCO reacted with MeOH at 60 °C to yield mixtures of fatty acid methyl esters (FAMEs) in the presence of 0.019% (w/w) WAS/WCO using the WAS (0.204 mol L-1, predetermined by potentiometric titration) from aluminum production by the Bayer process. The use of the new catalyst (WAS) resulted in a high yield of the products (greater than 99% yield).

Learning from the challenges of undertaking an evaluation of a multi-partner housing support initiative delivered within a hospital setting.

Objectives: We present learning from a mixed-methods evaluation of a housing support initiative for hospital inpatients. Study design: A mixed-methods process evaluation. Methods: A social housing provider delivered a housing support service in two hospitals (mental health unit and general hospital). Healthcare providers, the social housing provider and academic researchers designed and undertook a co-produced, mixed-methods process evaluation of the intervention. The evaluation included questionnaires, semi-structured interviews, analysis of routinely collected data and economic analysis. Despite commitment from the partners, the evaluation faced challenges. We reflect on the lessons learnt within our discussion paper. Results: Despite the commitment of the partners, we faced several challenges.We took an iterative approach to the design and processes of the evaluation to respond to arising challenges. Recruitment of service-users was more difficult than anticipated, requiring additional staff resources. Given the small-scale nature of the intervention, and the quality of data recorded in hospital records, the planned economic analysis was not feasible. Positive factors facilitating evaluation included involvement of staff delivering the intervention, as well as managers. Being able to offer payment to partner organisations for staff time also facilitated ongoing engagement. Conclusions: Multi-partner evaluations are useful, however, researchers and partners need to be prepared to take an iterative, resource intensive approach. Both availability and quality of routine data, and the resources required to support data collection, may limit feasibility of specific methods when evaluating small-scale cross-sector initiatives. Thus, this necessitates a flexible approach to design and analysis.

p62 overexpression induces TDP-43 cytoplasmic mislocalisation, aggregation and cleavage and neuronal death.

Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) that exist on a spectrum of neurodegenerative disease. A hallmark of pathology is cytoplasmic TDP-43 aggregates within neurons, observed in 97% of ALS cases and ~ 50% of FTLD cases. This mislocalisation from the nucleus into the cytoplasm and TDP-43 cleavage are associated with pathology, however, the drivers of these changes are unknown. p62 is invariably also present within these aggregates. We show that p62 overexpression causes TDP-43 mislocalisation into cytoplasmic aggregates, and aberrant TDP-43 cleavage that was dependent on both the PB1 and ubiquitin-associated (UBA) domains of p62. We further show that p62 overexpression induces neuron death. We found that stressors (proteasome inhibition and arsenic) increased p62 expression and that this shifted the nuclear:cytoplasmic TDP-43 ratio. Overall, our study suggests that environmental factors that increase p62 may thereby contribute to TDP-43 pathology in ALS and FTLD.

Shorter heels are linked with greater elastic energy storage in the Achilles tendon.

Previous research suggests that the moment arm of the m. triceps surae tendon (i.e., Achilles tendon), is positively correlated with the energetic cost of running. This relationship is derived from a model which predicts that shorter ankle moment arms place larger loads on the Achilles tendon, which should result in a greater amount of elastic energy storage and return. However, previous research has not empirically tested this assumed relationship. We test this hypothesis using an inverse dynamics approach in human subjects (n = 24) at speeds ranging from walking to sprinting. The spring function of the Achilles tendon was evaluated using specific net work, a metric of mechanical energy production versus absorption at a limb joint. We also combined kinematic and morphological data to directly estimate tendon stress and elastic energy storage. We find that moment arm length significantly determines the spring-like behavior of the Achilles tendon, as well as estimates of mass-specific tendon stress and elastic energy storage at running and sprinting speeds. Our results provide support for the relationship between short Achilles tendon moment arms and increased elastic energy storage, providing an empirical mechanical rationale for previous studies demonstrating a relationship between calcaneal length and running economy. We also demonstrate that speed and kinematics moderate tendon performance, suggesting a complex relationship between lower limb geometry and foot strike pattern.

Prospective contributions of biomass pyrolysis to China's 2050 carbon reduction and renewable energy goals.

Recognizing that bioenergy with carbon capture and storage (BECCS) may still take years to mature, this study focuses on another photosynthesis-based, negative-carbon technology that is readier to implement in China: biomass intermediate pyrolysis poly-generation (BIPP). Here we find that a BIPP system can be profitable without subsidies, while its national deployment could contribute to a 61% reduction of carbon emissions per unit of gross domestic product in 2030 compared to 2005 and result additionally in a reduction in air pollutant emissions. With 73% of national crop residues used between 2020 and 2030, the cumulative greenhouse gas (GHG) reduction could reach up to 8620 Mt CO2-eq by 2050, contributing 13-31% of the global GHG emission reduction goal for BECCS, and nearly 4555 Mt more than that projected for BECCS alone in China. Thus, China's BIPP deployment could have an important influence on achieving both national and global GHG emissions reduction targets.

Organic osmolytes increase expression of specific tight junction proteins in skin and alter barrier function in keratinocytes.

BACKGROUND: The epidermal barrier is important for water conservation, failure of which is evident in dry-skin conditions. Barrier function is fulfilled by the stratum corneum, tight junctions (TJs, which control extracellular water) and keratinocyte mechanisms, such as organic osmolyte transport, which regulate intracellular water homeostasis. Organic osmolyte transport by keratinocytes is largely unexplored and nothing is known regarding how cellular and extracellular mechanisms of water conservation may interact. OBJECTIVES: We aimed to characterize osmolyte transporters in skin and keratinocytes, and, using transporter inhibitors, to investigate whether osmolytes can modify TJs. Such modification would suggest a possible link between intracellular and extracellular mechanisms of water regulation in skin. METHODS: Immunostaining and quantitative polymerase chain reaction of organic osmolyte-treated organ-cultured skin were used to identify changes to organic osmolyte transporters, and TJ protein and gene expression. TJ functional assays were performed on organic osmolyte-treated primary human keratinocytes in culture. RESULTS: Immunostaining demonstrated the expression of transporters for betaine, taurine and myo-inositol in transporter-specific patterns. Treatment of human skin with either betaine or taurine increased the expression of claudin-1, claudin-4 and occludin. Osmolyte transporter inhibition abolished this response. Betaine and taurine increased TJ function in primary human keratinocytes in vitro. CONCLUSIONS: Treatment of skin with organic osmolytes modulates TJ structure and function, which could contribute to the epidermal barrier. This emphasizes a role for organic osmolytes beyond the maintenance of intracellular osmolarity. This could be harnessed to enhance topical therapies for diseases characterized by skin barrier dysfunction.

Prophylactic mesh augmentation using permanent synthetic mesh: outcomes of keyhole and Stapled Ostomy Reinforcement with Retromuscular Mesh techniques.

INTRODUCTION: Parastomal hernias (PSH) are the most common complication of stoma creation and can cause significant morbidity. We present a consecutive series of patients receiving prophylactic mesh augmentation (PMA) for prevention of PSH. METHODS: This retrospective review evaluates the efficacy and outcomes of PMA for PSH prevention, and retrospectively compares traditional keyhole PMA (tPMA) (n = 28) with a prophylactic Stapled Ostomy Reinforcement with Retromuscular Mesh technique (pSTORRM) (n = 24). RESULTS: PMA was performed in 52 cases between January 2015 and July 2018. All cases used a large-pore, non-coated, mid-weight polypropylene mesh placed in the retrorectus space. With a median follow-up of 16 mos, parastomal hernia was confirmed in 11.5% (n = 6), 5 of whom were symptomatic. patient-reported outcomes (PRO) indicated 6 additional patients with symptoms associated with PSH without clinical or radiographic confirmation. Patients had similar comorbidities and operative characteristics between tPMA and pSTORRM techniques, and no difference in a median follow-up. pSTORRM patients had fewer surgical site infections (8.3 vs 32.1%; p = 0.046) and occurrences (12.5 vs 46.4%; p = 0.015), and lower rate of PSH, though not statistically significant (4.2 vs 17.9%; p = 0.195). CONCLUSION: Permanent synthetic mesh placed as a sublay in the retromuscular space is safe and appears to decrease the risk of PSH formation after the creation of permanent stomas. A stapled technique may provide advantages over a traditional keyhole technique.

Excellent medium-term survival of an all-inside tensionable knotted suture device justifies repair of most meniscal tears encountered during reconstructive knee ligament surgery.

PURPOSE: All-inside meniscal repair devices have evolved to allow surgeons to undertake complex repairs in a timely and efficient manner. This is advantageous in active patients, where meniscus preservation is critical in preserving joint function and stability. The aim of the study was to evaluate the failure rate of all-inside meniscal repair performed in patients undergoing reconstructive ligament surgery using a particular meniscal repair device. METHODS: Patients were identified using a single-site prospectively maintained patient registry. Primary outcome was failure, defined as return to surgery with documented failure of repair. Complication rates and functional scores were also recorded. Patients in whom meniscal repair failure was identified were further assessed, to identify any common features. RESULTS: Over an 8-year period, 323 patients underwent meniscal repair at the time of ligament reconstruction, compared to 244 meniscectomies. Of these, 286 patients underwent repair using an all-inside suture device. One-hundred and twenty-seven repairs were to the medial meniscus only, 124 were lateral, and in 35 patients both menisci were repaired. Follow-up was to a median of 51.5 months. There were 31 (9.7%) failures reported at a median of 22 months post-operatively (IQR 13.5-41.5). Medial repair failures were seen more frequently than lateral (13.6% versus 5.6% OR 2.62 95% CI 1.17-5.88 p = 0.022). Failure of ACL reconstruction was associated with meniscal repair failure (OR 5.83 95% CI 1.55-21.95 p = 0.0039). Multi-ligament reconstruction was undertaken in 70/286 patients receiving meniscal repair and was not associated with failure (OR 1.3 95% CI 0.57-2.98 p = 0.51). Mode number of all-inside sutures used was 3 in both medial and lateral repairs (Range 1-9 lateral; 1-7 medial). CONCLUSIONS: All-inside repair is a safe and versatile technique which can be used in the majority of meniscal tears encountered during ligament reconstruction with excellent mid-term success. Failure is seen more commonly in medial sided repairs and with failure of ACL reconstruction. LEVEL OF EVIDENCE: IV.

Development, behaviour and sensory processing in Marshall-Smith syndrome and Malan syndrome: phenotype comparison in two related syndromes.

BACKGROUND: Ultrarare Marshall-Smith and Malan syndromes, caused by changes of the gene nuclear factor I X (NFIX), are characterised by intellectual disability (ID) and behavioural problems, although questions remain. Here, development and behaviour are studied and compared in a cross-sectional study, and results are presented with genetic findings. METHODS: Behavioural phenotypes are compared of eight individuals with Marshall-Smith syndrome (three male individuals) and seven with Malan syndrome (four male individuals). Long-term follow-up assessment of cognition and adaptive behaviour was possible in three individuals with Marshall-Smith syndrome. RESULTS: Marshall-Smith syndrome individuals have more severe ID, less adaptive behaviour, more impaired speech and less reciprocal interaction compared with individuals with Malan syndrome. Sensory processing difficulties occur in both syndromes. Follow-up measurement of cognition and adaptive behaviour in Marshall-Smith syndrome shows different individual learning curves over time. CONCLUSIONS: Results show significant between and within syndrome variability. Different NFIX variants underlie distinct clinical phenotypes leading to separate entities. Cognitive, adaptive and sensory impairments are common in both syndromes and increase the risk of challenging behaviour. This study highlights the value of considering behaviour within developmental and environmental context. To improve quality of life, adaptations to environment and treatment are suggested to create a better person-environment fit.

ALS-associated TBK1 variant p.G175S is defective in phosphorylation of p62 and impacts TBK1-mediated signalling and TDP-43 autophagic degradation.

Mutations affecting SQSTM1 coding for p62 and TANK-Binding Kinase 1 (TBK1) have been implicated in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). TBK1 is a serine-threonine kinase that regulates p62's activity as an autophagy receptor via phosphorylation and also has roles in neuroinflammatory signalling pathways. The mechanisms underlying ALS and FTLD pathogenesis as a result of TBK1 mutations are incompletely understood, however, loss of TBK1 function can lead to dysregulated autophagy and mitophagy. Here, we report that an ALS-associated TBK1 variant affecting the kinase domain, p.G175S, is defective in phosphorylation of p62 at Ser-403, a modification critical for regulating its ubiquitin-binding function, as well as downstream phosphorylation at Ser-349. Consistent with these findings, expression of p.G175S TBK1 was associated with decreased induction of autophagy compared to wild type and reduced degradation of the ALS-linked protein TDP-43. Expression of wild type TBK1 increased NF-κB signalling ~300 fold in comparison to empty vector cells, whereas p.G175S TBK1 was unable to promote NF-κB signalling above levels observed in empty vector transfected cells. We also noted a hitherto unknown role for TBK1 as a suppressor of oxidative stress (Nrf2) signalling and show that p.G175S TBK1 expressing cells lose this inhibitory function. Our data suggest that TBK1 ALS mutations may broadly impair p62-mediated cell signalling, which ultimately may reduce neuronal survival, in addition TDP-43 was not efficiently degraded, together these effects may contribute to TBK1 mutation associated ALS and FTLD pathogenesis.

Broadband, millimeter-wave antireflection coatings for large-format, cryogenic aluminum oxide optics.

We present two prescriptions for broadband ($ {\sim} 77 - 252\;{\rm GHz} $), millimeter-wave antireflection coatings for cryogenic, sintered polycrystalline aluminum oxide optics: one for large-format (700 mm diameter) planar and plano-convex elements, the other for densely packed arrays of quasi-optical elements-in our case, 5 mm diameter half-spheres (called "lenslets"). The coatings comprise three layers of commercially available, polytetrafluoroethylene-based, dielectric sheet material. The lenslet coating is molded to fit the 150 mm diameter arrays directly, while the large-diameter lenses are coated using a tiled approach. We review the fabrication processes for both prescriptions, then discuss laboratory measurements of their transmittance and reflectance. In addition, we present the inferred refractive indices and loss tangents for the coating materials and the aluminum oxide substrate. We find that at 150 GHz and 300 K the large-format coating sample achieves $ (97 \pm 2)\% $ transmittance, and the lenslet coating sample achieves $ (94 \pm 3)\% $ transmittance.

Biomethanation of invasive water hyacinth from eutrophic waters as a post weed management practice in the Dominican Republic: a developing country.

Anaerobic digestion of water hyacinth (Pontederia crassipes Mart.) from eutrophic water bodies could be a sustainable post weed management practice to generate bioenergy. Comparative analyses of the water quality, physicochemical characteristics, and biomethanation kinetics of water hyacinth from two sites with different water types (brackish versus freshwater) in the Ozama river, Dominican Republic, were conducted. Also, the energy produced from the anaerobic digestion and that consumed in harvesting was estimated. The highest non-structural components in the form of protein (18.8 ± 1.9%) and extractives (26.4 ± 0.1%) were found in brackish water hyacinth, whereas that from freshwater had the highest amount of holocellulose (41.2 ± 2.8%). Indicators of plant productivity, i.e., chlorophyll b and bulk density, were more than 30% higher in brackish than in freshwater hyacinth. The methane production rate in the digestion of water hyacinth from brackish water (22.5 N. L/kg VS added· day) was twice that from freshwater (10.0 N. L/kg VSadded· day). The higher nutrient content in the brackish water could have influenced the superior performance of water hyacinth from that source compared with that from freshwater. Overall, the maximum methane potential of the Ozama river water hyacinth was 399.2 ± 32.2 N. L CH4/kg VSadded. The estimated energy produced per ton of fresh biomass was 846.5 MJ, but only 57.9 MJ would be required for mechanical harvesting. The biomethanation of water hyacinth can mitigate weed management costs in developing countries.

Assessment of upgrading ability and limitations of slow co-pyrolysis: Case of olive mill wastewater sludge/waste tires slow co-pyrolysis.

Olive mill wastewater sludge (OMWS) and waste tires (WTs), abundant wastes in Tunisia, were used as feedstock in a slow co-pyrolysis pilot reactor to produce upgraded pyrolytic oil as an alternative fuel. Despite the improvement of some properties of the pyrolytic oil when waste tires were added in the feed blend, a negative synergy was observed in the yield of the oil compared with that of char. The characterization of oil samples showed synergetic interaction between OMWS and WTs during co-pyrolysis which led to a partial deoxygenation and resulted in reduction of viscosity and increase in the calorific value of the co-pyrolytic oils. However, the co-pyrolytic oil properties did not meet the requirements of commercial diesel and will need further improvement by effective standardization to meet marketable specifications. Compared with catalytic fast pyrolysis (CFP) followed by hydrodeoxygenation (HDO), OMWS/WTs slow co-pyrolysis showed some limitations but it can be considered as a simple, clean and cheap process upgrading technique for bio-oil production (∼40% lower in fixed capital investment and ∼30% lower in fuel selling price).