Reversible stimulus-responsive Cu(i) iodide pyridine coordination polymer

We present a structurally flexible copper–iodide–pyridine-based coordination polymer showing drastic variations in its electrical conductivity driven by temperature and sorption of acetic acid molecules. The dramatic effect on the electrical conductivity enables the fabrication of a simple and robust device for gas detection. X-ray diffraction studies and DFT calculations allow the rationalisation of these observations.We are thankful for support from MICINN (MAT2013-46753-C2-1-P, MAT2013-46502-C2-1/2-P and CTQ2011-26507), Eusko Jaurlaritza (S-PE13UN016) and Generalitat Valenciana PrometeoII/2014/076

Feasible combinatorial matrix theory

We show that the well-known Konig's Min-Max Theorem (KMM), a fundamental result in combinatorial matrix theory, can be proven in the first order theory \LA with induction restricted to $\Sigma_1^B$ formulas. This is an improvement over the standard textbook proof of KMM which requires $\Pi_2^B$ induction, and hence does not yield feasible proofs --- while our new approach does. \LA is a weak theory that essentially captures the ring properties of matrices; however, equipped with $\Sigma_1^B$ induction \LA is capable of proving KMM, and a host of other combinatorial properties such as Menger's, Hall's and Dilworth's Theorems. Therefore, our result formalizes Min-Max type of reasoning within a feasible framework

Excavations at Azoria, 2003–2004, Part 2: The Final Neolithic, Late Prepalatial, and Early Iron Age Occupation

This article constitutes the second of two reports on fieldwork conducted at Azoria in eastern Crete during the 2003 and 2004 excavation seasons. Evidence of Final Neolithic and Early Iron Age occupation and traces of Late Prepalatial activity were found underlying the Archaic civic buildings on the South Acropolis, particularly along the southwest terrace. The recovery of substantial Final Neolithic architectural and habitation remains contributes to our understanding of the 4th millennium in eastern Crete. Stratigraphic excavations have also clarified the spatial extent of the settlement from Late Minoan IIIC to the Late Geometric period, and brought to light evidence for the transition from the Early Iron Age to the Archaic period, and the transformation of the site in the 7th century B.C

Biallelic loss-of-function variants in PLD1 cause congenital right-sided cardiac valve defects and neonatal cardiomyopathy

Congenital heart disease is the most common type of birth defect, accounting for one-third of all congenital anomalies. Using whole-exome sequencing of 2718 patients with congenital heart disease and a search in GeneMatcher, we identified 30 patients from 21 unrelated families of different ancestries with biallelic phospholipase D1 (PLD1) variants who presented predominantly with congenital cardiac valve defects. We also associated recessive PLD1 variants with isolated neonatal cardiomyopathy. Furthermore, we established that p.1668F is a founder variant among Ashkenazi Jews (allele frequency of -.2%) and describe the phenotypic spectrum of PLD1-associated congenital heart defects. PLD1 missense variants were overrepresented in regions of the protein critical for catalytic activity, and, correspondingly, we observed a strong reduction in enzymatic activity for most of the mutant proteins in an enzymatic assay. Finally, we demonstrate that PLD1 inhibition decreased endothelial-mesenchymal transition, an established pivotal early step in valvulogenesis. In conclusion, our study provides a more detailed understanding of disease mechanisms and phenotypic expression associated with PLD1 loss of function.Genetics of disease, diagnosis and treatmen

Role of thrombospondin 1 in macrophage inflammation in dysferlin myopathy

Muscle inflammation can be a prominent feature in several muscular dystrophies. In dysferlin myopathy, it is mainly composed of macrophages. To understand the origin of inflammation in dysferlin-deficient muscle, we analyzed soluble factors involved in monocyte chemotaxis released by myoblasts and myotubes from control and dysferlinopathy patients using a transwell system. Dysferlin-deficient myotubes released more soluble factors involved in monocyte chemotaxis compared with controls (p < 0.001). Messenger RNA microarray analysis showed a 3.2-fold increase of thrombospondin 1 (TSP-1) expression in dysferlin-deficient myotubes. Retrotranscriptasepolymerase chain reaction analysis, ELISA, and immunohistochemistry confirmed these results. Dysferlin mRNA knockdown with short-interfering RNA in normal myogenic cells resulted in TSP-1 mRNA upregulation and increased chemotaxis. Furthermore, monocyte chemotaxis was decreased when TSP-1 was blocked by specific antibodies. In muscle biopsies from dysferlinopathy patients, TSP-1 expression was increased in muscle fibers but not in biopsies of patientswith other myopathies with inflammation; TSP-1 was seen in some macrophages in all samples analyzed. Taken together, the data demonstrate that dysferlin-deficient muscle upregulates TSP-1 in vivoand in vitro and indicate that endogenous chemotactic factors arecrucial to the sustained inflammatory process observed in dysferlinopathies. © 2010 by the American Association of Neuropathologists, Inc.This work was supported by the Beca del Fondo de Investigacion Sanitaria PI06/0455 and by the Centro de Investigacion Biomedica en Red sobre Enfermedades Neurodegenerativas.Peer Reviewe