99 research outputs found

    Correlative Light and Scanning X-Ray Scattering Microscopy of Healthy and Pathologic Human Bone Sections

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    Scanning small and wide angle X-ray scattering (scanning SWAXS) experiments were performed on healthy and pathologic human bone sections. Via crystallographic tools the data were transformed into quantitative images and as such compared with circularly polarized light (CPL) microscopy images. SWAXS and CPL images allowed extracting information of the mineral nanocrystalline phase embedded, with and without preferred orientation, in the collagen fibrils, mapping local changes at sub-osteon resolution. This favorable combination has been applied for the first time to biopsies of dwarfism syndrome and Paget's disease to shed light onto the cortical structure of natural bone in healthy and pathologic sections

    Identification of multiple integrin β1 homologs in zebrafish (Danio rerio)

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    BACKGROUND: Integrins comprise a large family of α,β heterodimeric, transmembrane cell adhesion receptors that mediate diverse essential biological functions. Higher vertebrates possess a single β1 gene, and the β1 subunit associates with a large number of α subunits to form the major class of extracellular matrix (ECM) receptors. Despite the fact that the zebrafish (Danio rerio) is a rapidly emerging model organism of choice for developmental biology and for models of human disease, little is currently known about β1 integrin sequences and functions in this organism. RESULTS: Using RT-PCR, complete coding sequences of zebrafish β1 paralogs were obtained from zebrafish embryos or adult tissues. The results show that zebrafish possess two β1 paralogs (β1–1 and β1–2) that have a high degree of identity to other vertebrate β1 subunits. In addition, a third, more divergent, β1 paralog is present (β1–3), which may have altered ligand-binding properties. Zebrafish also have other divergent β1-like transcripts, which are C-terminally truncated forms lacking the transmembrane and cytoplasmic domains. Together with β1–3 these truncated forms comprise a novel group of β1 paralogs, all of which have a mutation in the ADMIDAS cation-binding site. Phylogenetic and genomic analyses indicate that the duplication that gave rise to β1–1 and β1–2 occurred after the divergence of the tetrapod and fish lineages, while a subsequent duplication of the ancestor of β1–2 may have given rise to β1–3 and an ancestral truncated paralog. A very recent tandem duplication of the truncated β1 paralogs appears to have taken place. The different zebrafish β1 paralogs have varied patterns of temporal expression during development. β1–1 and β1–2 are ubiquitously expressed in adult tissues, whereas the other β1 paralogs generally show more restricted patterns of expression. CONCLUSION: Zebrafish have a large set of integrin β1 paralogs. β1–1 and β1–2 may share the roles of the solitary β1 subunit found in other vertebrates, whereas β1–3 and the truncated β1 paralogs may have acquired novel functions

    Comparative assessment of gasification based coal power plants with various CO2 capture technologies producing electricity and hydrogen

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    Seven different types of gasification-based coal conversion processes for producing mainly electricity and in some cases hydrogen (H2), with and without carbon dioxide (CO2) capture, were compared on a consistent basis through simulation studies. The flowsheet for each process was developed in a chemical process simulation tool “Aspen Plus”. The pressure swing adsorption (PSA), physical absorption (Selexol), and chemical looping combustion (CLC) technologies were separately analyzed for processes with CO2 capture. The performances of the above three capture technologies were compared with respect to energetic and exergetic efficiencies, and the level of CO2 emission. The effect of air separation unit (ASU) and gas turbine (GT) integration on the power output of all the CO2 capture cases is assessed. Sensitivity analysis was carried out for the CLC process (electricity-only case) to examine the effect of temperature and water-cooling of the air reactor on the overall efficiency of the process. The results show that, when only electricity production in considered, the case using CLC technology has an electrical efficiency 1.3% and 2.3% higher than the PSA and Selexol based cases, respectively. The CLC based process achieves an overall CO2 capture efficiency of 99.9% in contrast to 89.9% for PSA and 93.5% for Selexol based processes. The overall efficiency of the CLC case for combined electricity and H2 production is marginally higher (by 0.3%) than Selexol and lower (by 0.6%) than PSA cases. The integration between the ASU and GT units benefits all three technologies in terms of electrical efficiency. Furthermore, our results suggest that it is favorable to operate the air reactor of the CLC process at higher temperatures with excess air supply in order to achieve higher power efficiency

    Current status and future development of solvent-based carbon capture

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    Solvent-based carbon capture is the most commercially-ready technology for economically and sustainably reaching carbon emission reduction targets in the power sector. Globally, the technology has been deployed to deal with flue gases from large scale power plants and different carbon-intensive industries. The success of the technology is due to significant R&D activities on the process development and decades of industrial experience on acid gas removal processes from gaseous mixtures. In this paper, current status of PCC based on chemical absorption—commercial deployment and demonstration projects, analysis of different solvents and process configurations—is reviewed. Although some successes have been recorded in developing this technology, its commercialization has been generally slow as evidenced in the cancellation of high profile projects across the world. This is partly due to the huge cost burden of the technology and unpredictable government policies. Different research directions, namely new process development involving process intensification, new solvent development and a combination of both, are discussed in this paper as possible pathways for reducing the huge cost of the technology

    Transcriptional Profiling of Chondrodysplasia Growth Plate Cartilage Reveals Adaptive ER-Stress Networks That Allow Survival but Disrupt Hypertrophy

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    Metaphyseal chondrodysplasia, Schmid type (MCDS) is characterized by mild short stature and growth plate hypertrophic zone expansion, and caused by collagen X mutations. We recently demonstrated the central importance of ER stress in the pathology of MCDS by recapitulating the disease phenotype by expressing misfolding forms of collagen X (Schmid) or thyroglobulin (Cog) in the hypertrophic zone. Here we characterize the Schmid and Cog ER stress signaling networks by transcriptional profiling of microdissected mutant and wildtype hypertrophic zones. Both models displayed similar unfolded protein responses (UPRs), involving activation of canonical ER stress sensors and upregulation of their downstream targets, including molecular chaperones, foldases, and ER-associated degradation machinery. Also upregulated were the emerging UPR regulators Wfs1 and Syvn1, recently identified UPR components including Armet and Creld2, and genes not previously implicated in ER stress such as Steap1 and Fgf21. Despite upregulation of the Chop/Cebpb pathway, apoptosis was not increased in mutant hypertrophic zones. Ultrastructural analysis of mutant growth plates revealed ER stress and disrupted chondrocyte maturation throughout mutant hypertrophic zones. This disruption was defined by profiling the expression of wildtype growth plate zone gene signatures in the mutant hypertrophic zones. Hypertrophic zone gene upregulation and proliferative zone gene downregulation were both inhibited in Schmid hypertrophic zones, resulting in the persistence of a proliferative chondrocyte-like expression profile in ER-stressed Schmid chondrocytes. Our findings provide a transcriptional map of two chondrocyte UPR gene networks in vivo, and define the consequences of UPR activation for the adaptation, differentiation, and survival of chondrocytes experiencing ER stress during hypertrophy. Thus they provide important insights into ER stress signaling and its impact on cartilage pathophysiology
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