The novel missense mutation Met48Lys in FKBP22 changes its structure and functions.

Mutations in the FKBP14 gene encoding FKBP22 (FK506 Binding Protein 22 kDa) cause kyphoscoliotic Ehlers-Danlos Syndrome (kEDS). The first clinical report showed that a lack of FKBP22 protein due to mutations causing nonsense-mediated decay of the mRNA leads to a wide spectrum of clinical phenotypes including progressive kyphoscoliosis, joint hypermobility, hypotonia, hyperelastic skin, hearing loss and aortic rupture. Our previous work showed that these phenotypic features could be correlated with the functions of FKBP22, which preferentially binds to type III, VI and X collagens, but not to type I, II or V collagens. We also showed that FKBP22 catalyzed the folding of type III collagen through its prolyl isomerase activity and acted as a molecular chaperone for type III collagen. Recently, a novel missense mutation Met48Lys in FKBP22 was identified in a patient with kEDS. In this report, we expand the list of substrates of FKBP22 and also demonstrate that the Met48Lys mutation diminishes the activities of FKBP22, indicating that pathology can arise from absence of FKBP22, or partial loss of its function

Gestión de la investigación en la Universidad Nacional de Cuyo (1949-2010)

La Universidad Nacional de Cuyo, desde su origen, consideró a la investigación como una de sus funciones básicas. En tal sentido este libro surge como una propuesta para sistematizar, preservar y difundir información institucional, relacionada a la gestión del área específica. La Secretaría de Ciencia, Técnica y Posgrado de esta Casa de Estudios, a fines de 2009, invitó a participar de la iniciativa a quienes fueron responsables de esta tarea y a investigadores interesados en la historia de la institución, los que aportaron información y el valor de su experiencia. En el desarrollo de la obra se puede verificar claramente la evolución de estructuras y la definición de políticas científicas desarrolladas en la UNCuyo, ligadas a momentos históricos, políticas nacionales, locales e institucionales. Del relato de cada autor se observa que: planificación, mecanismos y estrategias de promoción de la investigación manifiestan una continuidad de esfuerzos cuyo objeto es incentivar la producción del conocimiento y su transferencia a las aulas y al medio. De la compiladora de la obra: Patricia Pons, Licenciada en Ciencias De la Educación de la Pontificia Universidad Católica Argentina Santa María de los Buenos Aires; se encuentra en la etapa de finalización de sus estudios de Maestría en Gestión de la Ciencia, la Técnica y la Innovación de la Universidad Nacional de General Sarmiento; Directora General de Ciencia y Técnica de la Secretaría de Ciencia Técnica y Posgrado de la Universidad Nacional de Cuyo

X-ray Pulsations from the region of the Supergiant Fast X-ray Transient IGR J17544-2619

Phase-targeted RXTE observations have allowed us to detect a transient 71.49 \pm 0.02 s signal that is most likely to be originating from the supergiant fast X-ray transient IGR J17544-2619. The phase-folded light curve shows a possible double-peaked structure with a pulsed flux of ~4.8*10^-12 erg cm^-2 s^-1 (3-10 keV). Assuming the signal to indicate the spin period of the neutron star in the system, the provisional location of IGR J17544-2619 on the Corbet diagram places the system within the classical wind-fed supergiant XRB region. Such a result illustrates the growing trend of supergiant fast X-ray transients to span across both of the original classes of HMXB in Porb - Pspin space.Comment: 7 pages, 6 figures, Accepted for publication in Astronomy and Astrophysics main journa

Ferromagnetism in Oriented Graphite Samples

We have studied the magnetization of various, well characterized samples of highly oriented pyrolitic graphite (HOPG), Kish graphite and natural graphite to investigate the recently reported ferromagnetic-like signal and its possible relation to ferromagnetic impurities. The magnetization results obtained for HOPG samples for applied fields parallel to the graphene layers - to minimize the diamagnetic background - show no correlation with the magnetic impurity concentration. Our overall results suggest an intrinsic origin for the ferromagnetism found in graphite. We discuss possible origins of the ferromagnetic signal.Comment: 11 figure

Measurement of the hadronic photon structure function F_{2}^{γ} at LEP2

The hadronic structure function of the photon F_{2}^{γ} (x, Q²) is measured as a function of Bjorken x and of the photon virtuality Q² using deep-inelastic scattering data taken by the OPAL detector at LEP at e⁺e⁻ centre-of-mass energies from 183 to 209 GeV. Previous OPAL measurements of the x dependence of F_{2}^{γ} are extended to an average Q² of 〈Q²〉=780 GeV² using data in the kinematic range 0.15<x<0.98. The Q² evolution of F_{2}^{γ} is studied for 12.1<〈Q²〉<780 GeV² using three ranges of x. As predicted by QCD, the data show positive scaling violations in F_{2}^{γ} with F_{2}^{γ} (Q²)/α = (0.08±0.02⁺⁰·⁰⁵_₀.₀₃) + (0.13±0.01⁺⁰·⁰¹_₀.₀₁) lnQ², where Q² is in GeV², for the central x region 0.10–0.60. Several parameterisations of F_{2}^{γ} are in qualitative agreement with the measurements whereas the quark-parton model prediction fails to describe the data

The Diagnostic Potential of Transition Region Lines under-going Transient Ionization in Dynamic Events

We discuss the diagnostic potential of high cadence ultraviolet spectral data when transient ionization is considered. For this we use high cadence UV spectra taken during the impulsive phase of a solar flares (observed with instruments on-board the Solar Maximum Mission) which showed excellent correspondence with hard X-ray pulses. The ionization fraction of the transition region ion O V and in particular the contribution function for the O V 1371A line are computed within the Atomic Data and Analysis Structure, which is a collection of fundamental and derived atomic data and codes which manipulate them. Due to transient ionization, the O V 1371A line is enhanced in the first fraction of a second with the peak in the line contribution function occurring initially at a higher electron temperature than in ionization equilibrium. The rise time and enhancement factor depend mostly on the electron density. The fractional increase in the O V 1371A emissivity due to transient ionization can reach a factor of 2--4 and can explain the fast response in the line flux of transition regions ions during the impulsive phase of flares solely as a result of transient ionization. This technique can be used to diagnostic the electron temperature and density of solar flares observed with the forth-coming Interface Region Imaging Spectrograph.Comment: 18 pages, 6 figure

The 2015-2016 outburst of the classical EXor V1118 Ori

After a quiescence period of about 10 years, the classical EXor source V1118 Ori has undergone an accretion outburst in 2015 September. The maximum brightness (DV > 4 mag) was reached in 2015 December and was maintained for several months. Since 2016 September, the source is in a declining phase. Photometry and low/ high-resolution spectroscopy were obtained with MODS and LUCI2 at the {\it Large Binocular Telescope}, with the facilities at the Asiago 1.22 and 1.82 m telescopes, and with GIANO at the {\it Telescopio Nazionale Galileo}. The spectra are dominated by emission lines of \hi\ and neutral metallic species. From line and continuum analysis we derive the mass accretion rate and its evolution during the outburst. Considering that extinction may vary between 1.5 and 2.9 mag, we obtain m_acc= 0.3$-$2.0 10$^{-8}$ m_sun/yr, in quiescence and m_acc= 0.2$-$1.9 10$^{-6}$ m_sun/yr, at the outburst peak. The Balmer decrement shape has been interpreted by means of line excitation models, finding that from quiescence to outburst peak, the electron density has increased from $\sim$ 2 10$^9$ cm$^{-3}$ to $\sim$ 4 10$^{11}$ cm$^{-3}$. The profiles of the metallic lines are symmetric and narrower than 100 km s$^{-1}$, while \hi\, and \hei\,\,lines show prominent wings extending up to $\pm$ 500 km s$^{-1}$. The metallic lines likely originate at the base of the accretion columns, where neutrals are efficiently shielded against the ionizing photons, while faster ionized gas is closer to the star. Outflowing activity is testified by the detection of a variable P Cyg-like profile of the H$\alpha$ and \hei\, 1.08\,$\mu$m lines.Comment: Accepted by Ap

Search for R-Parity Violating Decays of Scalar Fermions at LEP

A search for pair-produced scalar fermions under the assumption that R-parity is not conserved has been performed using data collected with the OPAL detector at LEP. The data samples analysed correspond to an integrated luminosity of about 610 pb-1 collected at centre-of-mass energies of sqrt(s) 189-209 GeV. An important consequence of R-parity violation is that the lightest supersymmetric particle is expected to be unstable. Searches of R-parity violating decays of charged sleptons, sneutrinos and squarks have been performed under the assumptions that the lightest supersymmetric particle decays promptly and that only one of the R-parity violating couplings is dominant for each of the decay modes considered. Such processes would yield final states consisting of leptons, jets, or both with or without missing energy. No significant single-like excess of events has been observed with respect to the Standard Model expectations. Limits on the production cross- section of scalar fermions in R-parity violating scenarios are obtained. Constraints on the supersymmetric particle masses are also presented in an R-parity violating framework analogous to the Constrained Minimal Supersymmetric Standard Model.Comment: 51 pages, 24 figures, Submitted to Eur. Phys. J.

Measurement of the ttbar Production Cross Section in ppbar Collisions at sqrt(s) = 1.96 TeV

We present a measurement of the top quark pair production cross section in ppbar collisions at sqrt(s)=1.96 TeV using 318 pb^{-1} of data collected with the Collider Detector at Fermilab. We select ttbar decays into the final states e nu + jets and mu nu + jets, in which at least one b quark from the t-quark decays is identified using a secondary vertex-finding algorithm. Assuming a top quark mass of 178 GeV/c^2, we measure a cross section of 8.7 +-0.9 (stat) +1.1-0.9 (syst) pb. We also report the first observation of ttbar with significance greater than 5 sigma in the subsample in which both b quarks are identified, corresponding to a cross section of 10.1 +1.6-1.4(stat)+2.0-1.3 (syst) pb.Comment: Accepted for publication in Physics Review Letters, 7 page

Colour reconnection in e+e- -> W+W- at sqrt(s) = 189 - 209 GeV

The effects of the final state interaction phenomenon known as colour reconnection are investigated at centre-of-mass energies in the range sqrt(s) ~ 189-209 GeV using the OPAL detector at LEP. Colour reconnection is expected to affect observables based on charged particles in hadronic decays of W+W-. Measurements of inclusive charged particle multiplicities, and of their angular distribution with respect to the four jet axes of the events, are used to test models of colour reconnection. The data are found to exclude extreme scenarios of the Sjostrand-Khoze Type I (SK-I) model and are compatible with other models, both with and without colour reconnection effects. In the context of the SK-I model, the best agreement with data is obtained for a reconnection probability of 37%. Assuming no colour reconnection, the charged particle multiplicity in hadronically decaying W bosons is measured to be (nqqch) = 19.38+-0.05(stat.)+-0.08 (syst.).Comment: 30 pages, 9 figures, Submitted to Euro. Phys. J.