BioTRIZ Suggests Radiative Cooling of Buildings Can Be Done Passively by Changing the Structure of Roof Insulation to Let Longwave Infrared Pass

This paper demonstrates the application of a design tool called BioTRIZ. Its developers claim that it can be used to access biological strategies for solving engineering problems. Our aim is to design a roof for hot climates that gets free cooling through radiant coupling with the sky. The insulation in a standard roof stops the sun and convection from warming the thermal mass. But it also restricts the mass＇s longwave view of the cool sky. Different solutions to this conflict are offered by BioTRIZ. The chosen solution is to replace the standard insulation component with an open cell honeycomb. The vertical cells would allow longwave radiation to pass, while arresting convection. The solutions offered by BioTRIZ＇s technological counterpart include no such changes in structure. It is estimated that the thermal mass in the biomimetic roof would remain on average 4.5℃ cooler than in a standard roof over a year in Riyadh, Saudi Arabia.     

Laminin-induced Clustering of Dystroglycan on Embryonic Muscle Cells: Comparison with Agrin-induced Clustering

The effect of laminin on the distribution of dystroglycan (DG) and other surface proteins was examined by fluorescent staining in cultures of muscle cells derived from Xenopus embryos. Western blotting confirmed that previously characterized antibodies are reactive in Xenopus. In control cultures, αDG, βDG, and laminin binding sites were distributed as microclusters (<1 μm² in area) over the entire dorsal surface of the muscle cells. Treatment with laminin induced the formation of macroclusters (1–20 μm²), accompanied by a corresponding decline in the density of the microclusters. With 6 nM laminin, clustering was apparent within 150 min and near maximal within 1 d. Laminin was effective at 30 pM, the lowest concentration tested. The laminin fragment E3, which competes with laminin for binding to αDG, inhibited laminin-induced clustering but did not itself cluster DG, thereby indicating that other portions of the laminin molecule in addition to its αDG binding domain are required for its clustering activity. Laminin-induced clusters also contained dystrophin, but unlike agrin-induced clusters, they did not contain acetylcholine receptors, utrophin, or phosphotyrosine, and their formation was not inhibited by a tyrosine kinase inhibitor. The results reinforce the notion that unclustered DG is mobile on the surface of embryonic muscle cells and suggest that this mobile DG can be trapped by at least two different sets of molecular interactions. Laminin self binding may be the basis for the laminin-induced clustering.     

Basement membrane assembly, stability and activities observed through a developmental lens.

Basement membranes are cell surface associated extracellular matrices containing laminins, type IV collagens, nidogens, perlecan, agrin, and other macromolecules. Biochemical and ultrastructural studies have suggested that basement membrane assembly and integrity is provided through multiple component interactions consisting of self-polymerizations, inter-component binding, and cell surface adhesions. Mutagenesis in vertebrate embryos and embryoid bodies have led to revisions of this model, providing evidence that laminins are essential for the formation of an initial polymeric scaffold of cell-attached matrix which matures in stability, ligand diversity, and functional complexity as additional matrix components are integrated into the scaffold. These studies also demonstrate that basement membrane components differentially promote cell polarization, organize and compartmentalize developing tissues, and maintain adult tissue function.     

The innervation of the uterus in Salamandra salamandra (L.) (Amphibia,Urodela)

The innervation of the uterus in the ovoviviparous urodele Salamandra salamandra was studied. In whole mount preparations of the thin-walled uterus of pregnant females, a dense adrenergic network was demonstrated using a modified glyoxylic acid fluorescence technique. Based on vesicle type and cytochemical reactivity after chromate/dichromate fixation for electron microscopy at least two types of neural process were distinguished and classified as adrenergic and cholinergic. Both types are preferentially situated above or between the smooth muscles of the uterine tissue. Adjacent to the muscles in the walls of arterioles mainly adrenergic fibers are seen. Using high performance liquid chromatography with electrochemical detection in tissue homogenates of uterus a considerable amount of noradrenaline could be identified. The significance of the dense innervation is discussed with respect to the function of the uterus during pregnancy and birth.     

Therapie mitochondrialer Erkrankungen

Although there have been major advances in understanding the pathogenesis of mitochondrial disorders, current treatment is largely supportive and there is still no cure. A variety of pharmacological agents, vitamins and cofactors, dietary modifications and interventions are under investigation, including attempts to bypass blocks in the respiratory chain, antioxidative effects, supplementation of deficiencies of specific compounds or the removal of noxious metabolites. There is currently no clear evidence supporting the use of any of these interventions. However, replacement of deficient metabolites or elimination of toxic molecules might be beneficial in specific disorders. Moreover, current data suggest that idebenone might be useful in Leber??s hereditary optic neuropathy (LHON). Only few randomized controlled trials had been conducted to date, and future controlled trials are strongly needed to establish the role of therapeutic approaches in homogeneous study populations. Gene therapy is a challenge, but promising experimental approaches are being pursued.     

Laminins in basement membrane assembly

The heterotrimeric laminins are a defining component of all basement membranes and self-assemble into a cell-associated network. The three short arms of the cross-shaped laminin molecule form the network nodes, with a strict requirement for one α, one β and one γ arm. The globular domain at the end of the long arm binds to cellular receptors, including integrins, α-dystroglycan, heparan sulfates and sulfated glycolipids. Collateral anchorage of the laminin network is provided by the proteoglycans perlecan and agrin. A second network is then formed by type IV collagen, which interacts with the laminin network through the heparan sulfate chains of perlecan and agrin and additional linkage by nidogen. This maturation of basement membranes becomes essential at later stages of embryo development.     

Gonosomale Mosaike

With an incidence of approximately 0.2?% in newborns gonosomal chromosome aberrations are of major relevance for clinical genetics. They frequently occur as numerical and/or structural gonosomal mosaicism. The correlation between genotype and phenotype is poor most probably due to different levels of mosaicism in different tissues and they represent a great challenge especially in prenatal diagnostics, requiring genetic counseling by an experienced clinical geneticist. Postnatally, gonosomal mosaicism in females often leads to the clinical symptoms of Turner’s syndrome (especially short stature and infertility) with an potentially increased risk for gonadoblastoma if an XY cell line is present. In males with Klinefelter’s syndrome mosaicism is also frequent (up to 20?%). Mosaicism in 47,XYY and 47,XXX karyotypes is rarely reported perhaps due to the innocuous phenotype seldom being an indication for chromosome analysis.     

Heredit?re Fiebersyndrome

Familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), and tumour necrosis factor (TNF) receptor-1-associated periodic syndrome (TRAPS) are monogenic disorders included under the term??hereditary fever syndromes??. These diseases are characterized by recurrent episodes of fever and inflammation and arise from mutations of genes regulating the innate immune system. The present review describes the clinical and genetic spectrum of hereditary fever syndromes, which are of importance for genetic counseling.