A new seismic isolation device based on tribological smooth rocking (TROCKSISD)

In the field of seismic risk mitigation of art objects, an innovative isolation device is here illus-trated. The device, called TROCKSISD (Tribological ROCKing Seismic ISolation Device), couples multiple components to dissipate energy and control smooth rocking: spherical con-tact surfaces with frictional layers, elastic springs ensuring re-centering and viscous elastic dampers. The conceptual idea is described and the equations of motions of the two degrees of freedom system presented, discussing the performance-based structural behaviour and the mechanical/geometric parameters involved. Moreover, dynamic analyses are performed to understand the role of the frictional layers with respect to the peripheral dampers in the miti-gation of motion under spectrum compatible seismic records. The results in terms of rocking spectra are presented and comparisons with the equivalent single degree of freedom system without the isolation device are made

Automated generation of flat tileable patterns and 3D reduced model simulation

The computational fabrication community is developing an increasing interest in the use of patterned surfaces, which can be designed to show ornamental and unconventional aesthetics or to perform as a proper structural material with a wide range of features. Geometrically designing and controlling the deformation capabilities of these patterns in response to external stimuli is a complex task due to the large number of variables involved. This paper introduces a method for generating sets of tileable and exchangeable flat patterns as well as a model-reduction strategy that enables their mechanical simulation at interactive rates. This method is included in a design pipeline that aims to turn any general flat surface into a pattern tessellation, which is able to deform under a given loading scenario. To validate our approach, we apply it to different contexts, including real-scale 3D printed specimens, for which we compare our results with the ones provided by a ground-truth solver

Concept and cable-tensioning optimization of post-tensioned shells made of structural glass

Shells made of structural glass are charming objects from both the aesthetics and the engineering point of view. However, they pose two signicant challenges: the rst one is to assure adequate safety and redundancy concerning possible global collapse; the second one is to guarantee the economy for replacing collapsed components. To address both requirements, this research explores a novel concept where triangular panels of structural glass are both post-tensioned and reinforced to create 3D free-form systems. Hence, the ligree steel truss, made of edges reinforcements, is sized in performance-based perspective to bear at least the weight of all panels in the occurrence of simultaneous cracks (worst-case scenario). The panels are post-tensioned using a set of edge-aligned cables that add benecial compressive stress on the surface. The cable placement and pre-loads are optimized to minimize the tensile stress acting on the shell and match the manufacturing constraints. These shells optimize material usage by providing not only a transparent and fascinating building separation but also load-bearing capabilities. Visual and structural lightness are improved to grid shell competitors

Conception and parametric design workflow for a timber large-spanned reversible grid shell to shelter the archaeological site of the Roman Shipwrecks in Pisa

Reciprocal structures or nexorade are composed by the assembling of groups of three or more beams mutually connected by mono-lateral T joints in a way that any relative movement is suppressed. This kind of structures can be easily built in relatively unprepared sites, dismantled, transported and re-used even by not specialized handcraft. For these reasons, reciprocal structures have been widely used in the past for military purposes, and nowadays they seem to satisfy very well the different requirements of a quick and temporary shelter of a large archaeological area when they are shaped as grid shells. This paper proposes the design of a reversible, reciprocal framed grid shell to shelter the archaeological site of the Roman Shipwrecks in Pisa. The structure must protect excavations and archaeologists from the weather and provide an easy access to visitors. Additionally, it must allow for easy disassembling and moving to another site. The design choices aim at optimizing both structural efficiency and esthetical qualities. A parametric workflow for both the form finding and the digital fabrication processes has been developed, and a prototype of accommodative steel T-joint for timber reciprocal beams has been realized. Finally, a model using CNC-cutting tested the structural feasibility of such a design approach

Integrated computational framework for the design and fabrication of bending-active structures made from flat sheet material

This paper introduces an integrated computational design framework for the design and realization of arbitrarily-curved bending-active architectural structures. The developed framework consists of a series of methods that enable the production of a complex 3D structures composed of a set of flat 2D panels whose mechanical properties are locally tuned by varying the shape of embedded spiraling patterns. The resulting panels perform as variable stiffness elements, and they are optimized to match a desired target shape once assembled together. The presented framework includes all the steps for the physical delivery of architectural objects, including conception, static assessment, and digital fabrication. The developed framework has been applied to an architectural scale prototype, which demonstrates the potential of integrating architectural design, computational simulation, structural engineering, and digital fabrication, opening up several possible novel applications in the building sector.</p

Case Report: Retracing Atypical Development: A Preserved Speech Variant of Rett Syndrome

The subject of the present study is the development of a girl with the preserved speech variant of Rett disorder. Our data are based on detailed retrospective and prospective video analyses. Despite achieving developmental milestones, movement quality was already abnormal during the girl's first half year of life. In addition, early hand stereotypies, idiosyncratic vocalizations, asymmetric eye opening, and abnormal facial expressions are early signs proving that this variant of the Rett complex, too, manifests itself within the first months of life

Expression profiling of clonal lymphocyte cell cultures from Rett syndrome patients

BACKGROUND: More than 85% of Rett syndrome (RTT) patients have heterozygous mutations in the X-linked MECP2 gene which encodes methyl-CpG-binding protein 2, a transcriptional repressor that binds methylated CpG sites. Because MECP2 is subject to X chromosome inactivation (XCI), girls with RTT express either the wild type or mutant MECP2 in each of their cells. To test the hypothesis that MECP2 mutations result in genome-wide transcriptional deregulation and identify its target genes in a system that circumvents the functional mosaicism resulting from XCI, we performed gene expression profiling of pure populations of untransformed T-lymphocytes that express either a mutant or a wild-type allele. METHODS: Single T lymphocytes from a patient with a c.473C>T (p.T158M) mutation and one with a c.1308-1309delTC mutation were subcloned and subjected to short term culture. Gene expression profiles of wild-type and mutant clones were compared by oligonucleotide expression microarray analysis. RESULTS: Expression profiling yielded 44 upregulated genes and 77 downregulated genes. We compared this gene list with expression profiles of independent microarray experiments in cells and tissues of RTT patients and mouse models with Mecp2 mutations. These comparisons identified a candidate MeCP2 target gene, SPOCK1, downregulated in two independent microarray experiments, but its expression was not altered by quantitative RT-PCR analysis on brain tissues from a RTT mouse model. CONCLUSION: Initial expression profiling from T-cell clones of RTT patients identified a list of potential MeCP2 target genes. Further detailed analysis and comparison to independent microarray experiments did not confirm significantly altered expression of most candidate genes. These results are consistent with other reported data

Molecular Cloning and Copy Number Variation of a Ferritin Subunit (Fth1) and Its Association with Growth in Freshwater Pearl Mussel Hyriopsis cumingii

Iron is one of the most important minor elements in the shells of bivalves. This study was designed to investigate the involvement of ferritin, the principal protein for iron storage, in shell growth. A novel ferritin subunit (Fth1) cDNA from the freshwater pearl mussel (Hyriopsis cumingii) was isolated and characterized. The complete cDNA contained 822 bp, with an open reading frame (ORF) of 525 bp, a 153 bp 5′ untranslated region (UTR) and a 144 bp 3′ UTR. The complete genomic DNA was 4125 bp, containing four exons and three introns. The ORF encoded a protein of 174 amino acids without a signal sequence. The deduced ferritin contained a highly conserved motif for the ferroxidase center comprising seven residues of a typical vertebrate heavy-chain ferritin. It contained one conserved iron associated residue (Try27) and iron-binding region signature 1 residues. The mRNA contained a 27 bp iron-responsive element with a typical stem-loop structure in the 5′-UTR position. Copy number variants (CNVs) of Fth1 in two populations (PY and JH) were detected using quantitative real-time PCR. Associations between CNVs and growth were also analyzed. The results showed that the copy number of the ferritin gene of in the diploid genome ranged from two to 12 in PY, and from two to six in JH. The copy number variation in PY was higher than that in JH. In terms of shell length, mussels with four copies of the ferritin gene grew faster than those with three copies (P<0.05), suggesting that CNVs in the ferritin gene are associated with growth in shell length and might be a useful molecular marker in selective breeding of H. cumingii

Ex Vivo Treatment with a Novel Synthetic Aminoglycoside NB54 in Primary Fibroblasts from Rett Syndrome Patients Suppresses MECP2 Nonsense Mutations

BACKGROUND: Nonsense mutations in the X-linked methyl CpG-binding protein 2 (MECP2) comprise a significant proportion of causative MECP2 mutations in Rett syndrome (RTT). Naturally occurring aminoglycosides, such as gentamicin, have been shown to enable partial suppression of nonsense mutations related to several human genetic disorders, however, their clinical applicability has been compromised by parallel findings of severe toxic effects. Recently developed synthetic NB aminoglycosides have demonstrated significantly improved effects compared to gentamicin evident in substantially higher suppression and reduced acute toxicity in vitro. RESULTS: We performed comparative study of suppression effects of the novel NB54 and gentamicin on three MECP2 nonsense mutations (R294X, R270X and R168X) common in RTT, using ex vivo treatment of primary fibroblasts from RTT patients harboring these mutations and testing for the C-terminal containing full-length MeCP2. We observed that NB54 induces dose-dependent suppression of MECP2 nonsense mutations more efficiently than gentamicin, which was evident at concentrations as low as 50 µg/ml. NB54 read-through activity was mutation specific, with maximal full-length MeCP2 recovery in R168X (38%), R270X (27%) and R294X (18%). In addition, the recovered MeCP2 was translocated to the cell nucleus and moreover led to parallel increase in one of the most important MeCP2 downstream effectors, the brain derived neurotrophic factor (BDNF). CONCLUSION: Our findings suggest that NB54 may induce restoration of the potentially functional MeCP2 in primary RTT fibroblasts and encourage further studies of NB54 and other rationally designed aminoglycoside derivatives as potential therapeutic agents for nonsense MECP2 mutations in RTT