A CD2AP mutation associated with focal segmental glomerulosclerosis in young adulthood

Mutations in CD2-associated protein (CD2AP) have been identified in patients with focal segmental glomerulosclerosis (FSGS); however, reports of CD2AP mutations remain scarce. We performed Sanger sequencing in a patient with steroid-resistant FSGS and identified a heterozygous CD2AP mutation (p.T374A, c.1120 A > G). Our patient displayed mild cognitive decline, a phenotypic characteristic not previously associated with CD2AP-associated FSGS. His proteinuria was remarkably reduced by treatment with cyclosporine A. Our findings expand the genetic spectrum of CD2AP-associated disorders and broaden the associated phenotype with the co-occurrence of cognitive decline. Our case shows that cyclosporin A is a treatment option for CD2AP-associated nephropathy

Gauge copies in the Landau-DeWitt gauge: a background invariant restriction

The Landau background gauge, also known as the Landau-DeWitt gauge, has found renewed interest during the past decade given its usefulness in accessing the confinement-deconfinement transition via the vacuum expectation value of the Polyakov loop, describable via an appropriate background. In this Letter, we revisit this gauge from the viewpoint of it displaying gauge (Gribov) copies. We generalize the Gribov-Zwanziger effective action in a BRST and background invariant way; this action leads to a restriction on the allowed gauge fluctuations, thereby eliminating the infinitesimal background gauge copies. The explicit background invariance of our action is in contrast with earlier attempts to write down and use an effective Gribov-Zwanziger action. It allows to address certain subtleties arising in these earlier works, such as a spontaneous and thus spurious Lorentz symmetry breaking, something which is now averted.Comment: 14 pages. v2: version to appear in Phys.Lett.B, with minor modifications and extra reference

Approximate tight-binding sum rule for the superconductivity related change of c-axis kinetic energy in multilayer cuprate superconductors

We present an extension of the c-axis tight-binding sum rule discussed by Chakravarty, Kee, and Abrahams [Phys. Rev. Lett. 82, 2366 (1999)] that applies to multilayer high-Tc cuprate superconductors (HTCS) and use it to estimate--from available infrared data--the change below Tc of the c-axis kinetic energy, Hc, in YBa2Cu3O(7-delta) (delta=0.45,0.25,0.07), Bi2Sr2CaCu2O8, and Bi2Sr2Ca2Cu3O10. In all these compounds Hc decreases below Tc and except for Bi2Sr2CaCu2O8 the change of Hc is of the same order of magnitude as the condensation energy. This observation supports the hypothesis that in multilayer HTCS superconductivity is considerably amplified by the interlayer tunnelling mechanism.Comment: 6 pages, 2 figure

Correlation between the Josephson coupling energy and the condensation energy in bilayer cuprate superconductors

We review some previous studies concerning the intra-bilayer Josephson plasmons and present new ellipsometric data of the c-axis infrared response of almost optimally doped Bi_{2}Sr_{2}CaCu_{2}O_{8}. The c-axis conductivity of this compound exhibits the same kind of anomalies as that of underdoped YBa_{2}Cu_{3}O_{7-delta}. We analyze these anomalies in detail and show that they can be explained within a model involving the intra-bilayer Josephson effect and variations of the electric field inside the unit cell. The Josephson coupling energies of different bilayer compounds obtained from the optical data are compared with the condensation energies and it is shown that there is a reasonable agreement between the values of the two quantities. We argue that the Josephson coupling energy, as determined by the frequency of the intra-bilayer Josephson plasmon, represents a reasonable estimate of the change of the effective c-axis kinetic energy upon entering the superconducting state. It is further explained that this is not the case for the estimate based on the use of the simplest ``tight-binding'' sum rule. We discuss possible interpretations of the remarkable agreement between the Josephson coupling energies and the condensation energies. The most plausible interpretation is that the interlayer tunneling of the Cooper pairs provides the dominant contribution to the condensation energy of the bilayer compounds; in other words that the condensation energy of these compounds can be accounted for by the interlayer tunneling theory. We suggest an extension of this theory, which may also explain the high values of T_{c} in the single layer compounds Tl_{2}Ba_{2}CuO_{6} and HgBa_{2}CuO_{4}, and we make several experimentally verifiable predictions.Comment: 16 pages (including Tables) and 7 figures; accepted for publication in Physical Review

MESoR - Management and exploitation of solar resource knowledge

CD-ROMKnowledge of the solar resource is essential for the planning and operation of solar energy systems. A number of data bases giving information on solar resources have been developed over the past years. The result is a fragmentation of services each having each own mechanism of access and all are giving different results due to different methods, input data and base years. The project MESoR, co-funded by the European Commission, reduces the associated uncertainty by setting up standard benchmarking rules and measures for comparing the data bases, user guidance to the application of resource data and unifying access to various data bases

Clarifying status of DNNs as models of human vision

On several key issues we agree with the commentators. Perhaps most importantly, everyone seems to agree that psychology has an important role to play in building better models of human vision, and (most) everyone agrees (including us) that deep neural networks (DNNs) will play an important role in modelling human vision going forward. But there are also disagreements about what models are for, how DNN-human correspondences should be evaluated, the value of alternative modelling approaches, and impact of marketing hype in the literature. In our view, these latter issues are contributing to many unjustified claims regarding DNN-human correspondences in vision and other domains of cognition. We explore all these issues in this response

The ALICE experiment at the CERN LHC

ALICE (A Large Ion Collider Experiment) is a general-purpose, heavy-ion detector at the CERN LHC which focuses on QCD, the strong-interaction sector of the Standard Model. It is designed to address the physics of strongly interacting matter and the quark-gluon plasma at extreme values of energy density and temperature in nucleus-nucleus collisions. Besides running with Pb ions, the physics programme includes collisions with lighter ions, lower energy running and dedicated proton-nucleus runs. ALICE will also take data with proton beams at the top LHC energy to collect reference data for the heavy-ion programme and to address several QCD topics for which ALICE is complementary to the other LHC detectors. The ALICE detector has been built by a collaboration including currently over 1000 physicists and engineers from 105 Institutes in 30 countries. Its overall dimensions are 161626 m3 with a total weight of approximately 10 000 t. The experiment consists of 18 different detector systems each with its own specific technology choice and design constraints, driven both by the physics requirements and the experimental conditions expected at LHC. The most stringent design constraint is to cope with the extreme particle multiplicity anticipated in central Pb-Pb collisions. The different subsystems were optimized to provide high-momentum resolution as well as excellent Particle Identification (PID) over a broad range in momentum, up to the highest multiplicities predicted for LHC. This will allow for comprehensive studies of hadrons, electrons, muons, and photons produced in the collision of heavy nuclei. Most detector systems are scheduled to be installed and ready for data taking by mid-2008 when the LHC is scheduled to start operation, with the exception of parts of the Photon Spectrometer (PHOS), Transition Radiation Detector (TRD) and Electro Magnetic Calorimeter (EMCal). These detectors will be completed for the high-luminosity ion run expected in 2010. This paper describes in detail the detector components as installed for the first data taking in the summer of 2008

Modelling probability matching as a Bayesian sampling process

The mechanisms underpinning probability matching remain a disputed topic. Among common explanations of the effect is that people employ a win-stay, lose-shift (WSLS) strategy. We suggest an alternative framing of probability matching as the result of a Bayesian sampling process involving simulating a mental sequence of possible outcomes. In three within-subject tasks, we presented people with information about a six-sided die with four sides of one colour and two of another. Two of them involved predicting the next outcome in a series of die rolls, with and without feedback. The third explicitly asked participants to mentally generate sequences of rolls from the die. The patterns of autocorrelations in responses, the absence of an effect of feedback on the next response, and the elevated proportion of maximising responses on the first trial in all conditions are all consistent with a Bayesian sampling model but contradict the WSLS account of probability matching