Comparison of Different Finite Element Models for the Transient Dynamic Analysis of Laminated Glass for Structural Applications

Commercial laminated glass is usually composed of two glass layers and an interlayer of PVB. The viscoelastic behaviour of the PVB layer has to be taken into account when dealing with dynamic loads. This paper shows different Finite Element (FE) models developed to characterize laminated glass. Results are contrasted with data from different reference test cases. First, a quasi-static test is reproduced with a 2d model through a transient analysis. In addition, flexural modes of vibration in a free-free test configuration have been analysed using 2d as well as 3d FE models. Apart from using transient analysis in order to simulate the dynamic behaviour of laminated glass, an iterative procedure has been employed which permits to identify the correct value of the shear modulus of the PVB layer for each mode in an eigenvalue analysis

CIBERER : Spanish national network for research on rare diseases: A highly productive collaborative initiative

Altres ajuts: Instituto de Salud Carlos III (ISCIII); Ministerio de Ciencia e Innovación.CIBER (Center for Biomedical Network Research; Centro de Investigación Biomédica En Red) is a public national consortium created in 2006 under the umbrella of the Spanish National Institute of Health Carlos III (ISCIII). This innovative research structure comprises 11 different specific areas dedicated to the main public health priorities in the National Health System. CIBERER, the thematic area of CIBER focused on rare diseases (RDs) currently consists of 75 research groups belonging to universities, research centers, and hospitals of the entire country. CIBERER's mission is to be a center prioritizing and favoring collaboration and cooperation between biomedical and clinical research groups, with special emphasis on the aspects of genetic, molecular, biochemical, and cellular research of RDs. This research is the basis for providing new tools for the diagnosis and therapy of low-prevalence diseases, in line with the International Rare Diseases Research Consortium (IRDiRC) objectives, thus favoring translational research between the scientific environment of the laboratory and the clinical setting of health centers. In this article, we intend to review CIBERER's 15-year journey and summarize the main results obtained in terms of internationalization, scientific production, contributions toward the discovery of new therapies and novel genes associated to diseases, cooperation with patients' associations and many other topics related to RD research

Novel homozygous variants in theSERPING1gene in two Turkish families with hereditary angioedema of recessive inheritance

Lopez Lera, Alberto/0000-0002-9596-6910; GULBAHAR, OKAN/0000-0002-5341-9070; Lopez-Trascasa, Margarita/0000-0001-8594-282XWOS: 000547270600001PubMed: 32445210Hereditary angioedema as a result of deficiency of the C1 inhibitor (HAE-C1INH; MIM# 106100) is a rare autosomal disorder and affected individuals are generally heterozygous for dominant negative variants in theSERPING1gene. Homozygosity forSERPING1pathogenic variants was long considered to be embryonically lethal; however, five nonrelated families with a recessive HAE pattern have been described in the last decade. in this report, we functionally characterized two newly reported nonrelated, consanguineous families with a recessive presentation of HAE attributed toSERPING1variants in the reactive center loop (family D; S438F) and gate (family A; I379T) regions. S438F heterozygotes (family D) showed variable levels of intact 105-kDa and cleaved/inactive 96-kDa isoforms of C1INH, whereas their homozygous relative presented only the 96-kDa band. Functional studies showed that S438F reduced C1INH interaction with target proteases in heterozygous (C1s, 32-38% of controls and FXIIa, 28-35% of controls) and homozygous (C1s, 18-24% of controls and FXIIa, 4-8% of controls) carriers, which is consistent with the more severe presentation of HAE in the family and decreased C1q levels in homozygous patients. By contrast, plasma C1INH from I379T heterozygotes (family A) showed normal C1INH/C1s binding (84-94% of controls) and no significant reduction in C1INH/FXIIa complexes (50-70% of controls). However, the homozygote failed to inhibit both C1s (25-42% of controls) and FXIIa (14-18% of controls). This profile is concordant with the less severe presentation of HAE in the family and the conserved C4 and C1q levels in heterozygous and homozygous patients.[ER19P7AC7541/2019]We thank Dr Alper Ozdemir, Arda Kula, Suat Hopanci and Betul Hopanci for obtaining the patient's serum samples. Alberto Lopez Lera is supported by grant ER19P7AC7541/2019 from Centre for Biomedical Network Research or Rare Diseases (CIBERER)