hCALCRL mutation causes autosomal recessive nonimmune hydrops fetalis with lymphatic dysplasia

We report the first case of nonimmune hydrops fetalis (NIHF) associated with a recessive, in-frame deletion of V205 in the G protein–coupled receptor, Calcitonin Receptor-Like Receptor (hCALCRL). Homozygosity results in fetal demise from hydrops fetalis, while heterozygosity in females is associated with spontaneous miscarriage and subfertility. Using molecular dynamic modeling and in vitro biochemical assays, we show that the hCLR(V205del) mutant results in misfolding of the first extracellular loop, reducing association with its requisite receptor chaperone, receptor activity modifying protein (RAMP), translocation to the plasma membrane and signaling. Using three independent genetic mouse models we establish that the adrenomedullin–CLR–RAMP2 axis is both necessary and sufficient for driving lymphatic vascular proliferation. Genetic ablation of either lymphatic endothelial Calcrl or nonendothelial Ramp2 leads to severe NIHF with embryonic demise and placental pathologies, similar to that observed in humans. Our results highlight a novel candidate gene for human congenital NIHF and provide structure–function insights of this signaling axis for human physiology

Angular momenta of fission fragments in the α-accompanied fission of

For the first time, average angular momenta of the ternary fission fragments 100, 102Zr, 106Mo, 144, 146Ba and 138, 140, 142Xe from the α-accompanied fission of 252Cf were obtained from relative intensities of prompt γ-ray transitions with the use of the statistical model calculation. Average values of the angular momenta were compared with the corresponding values for the same fission fragments from the binary fission of 252Cf. Results indicate the presence of a decreasing trend in the average values of angular momenta induced in ternary fission fragments compared to the same binary fission fragments. On the average, the total angular momentum extracted for ternary fission fragments is ∼1.4 ℏ lower than in binary fission. Consequently, results indicate that the mechanism of the ternary α-particles emission may directly effect an induction of angular momenta of fission fragments, and possible scenarios of such mechanisms are discussed. Further, the dependence of the angular momenta of 106Mo and 140Xe on the number of emitted neutrons from correlated pairs of primary fragments was obtained also showing a decreasing dependence of average angular momenta with increasing number of emitted neutrons. Consequences are briefly discussed

Turkey: A Regional Power Facing a Changing International System

Turkey's foreign policy activism in recent decades, characterized by an ambitious regional agenda and visibility in global affairs, has generated a lively debate. This paper attempts to position Turkey in modern-day international relations, in order to develop an analytical framework capable of theorizing its regional and global-level activism coherently. It thus proposes to conceptualize Turkey as a regional power and argues that such actors' behavior can be analyzed with reference to three interrelated variables: the nature of regional order, their behavioral attitudes, and the interactions at the regional-global nexus