Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency

Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with defective complement regulation caused by multiple factors. We previously described the deficiency of factor H-related proteins CFHR1 and CFHR3 as predisposing factor for aHUS. Here we identify in an extended cohort of 147 aHUS patients that 16 juvenile individuals (ie, 11%) who either lacked the CFHR1/CFHR3 completely (n = 14) or showed extremely low CFHR1/CFHR3 plasma levels (n = 2) are positive for factor H (CFH) autoantibodies. The binding epitopes of all 16 analyzed autoantibodies were localized to the C-terminal recognition region of factor H, which represents a hot spot for aHUS mutations. Thus we define a novel subgroup of aHUS, termed DEAP HUS (deficiency of CFHR proteins and CFH autoantibody positive) that is characterized by a combination of genetic and acquired factors. Screening for both factors is obviously relevant for HUS patients as reduction of CFH autoantibody levels represents a therapeutic option

High resolution spectroscopy of Pluto's atmosphere: detection of the 2.3 μ\mum CH4_4 bands and evidence for carbon monoxide

The goal is to determine the composition of Pluto's atmosphere and to constrain the nature of surface-atmosphere interactions. We perform high--resolution spectroscopic observations in the 2.33--2.36 $\mu$m range, using CRIRES at the VLT. We obtain (i) the first detection of gaseous methane in this spectral range, through lines of the $\nu_3$ + $\nu_4$ and $\nu_1$ + $\nu_4$ bands (ii) strong evidence (6-$\sigma$ confidence) for gaseous CO in Pluto. For an isothermal atmosphere at 90 K, the CH$_4$ and CO column densities are 0.75 and 0.07 cm-am, within factors of 2 and 3, respectively. Using a physically--based thermal structure model of Pluto's atmosphere also satisfying constraints from stellar occultations, we infer CH$_4$ and CO mixing ratios q$_{CH_4}$= 0.6$^{+0.6}_{-0.3}$% (consistent with results from the 1.66 $\mu$m range) and q$_{CO}$ = 0.5$^{+1}_{-0.25}$$\times10^{-3}$. The CO atmospheric abundance is consistent with its surface abundance. As for Triton, it is probably controlled by a thin, CO-rich, detailed balancing layer resulting from seasonal transport and/or atmospheric escape.Comment: Astronomy and Astrophysics Letters, in pres

Io: IUE observations of its atmosphere and the plasma torus

Two of the main components of the atmosphere of Io, neutral oxygen and sulfur, were detected with the IUE. Four observations yield brightnesses that are similar, regardless of whether the upstream or the downstream sides of the torus plasma flow around Io is observed. A simple model requires the emissions to be produced by the interaction of O and S columns in the exospheric range with 2 eV electrons. Cooling of the 5 eV torus electrons is required prior to their interaction with the atmosphere of Io. Inconsistencies in the characteristics of the spectra that cannot be accounted for in this model require further analysis with improved atomic data. The Io plasma torus was monitored with the IUE. The long-term stability of the warm torus is established. The observed brightnesses were analyzed using a model of the torus, and variations of less than 30 percent in the composition are observed, the quantitative results being model dependent

First High Contrast Imaging Using a Gaussian Aperture Pupil Mask

Placing a pupil mask with a gaussian aperture into the optical train of current telescopes represents a way to attain high contrast imaging that potentially improves contrast by orders of magnitude compared to current techniques. We present here the first observations ever using a gaussian aperture pupil mask (GAPM) on the Penn State near-IR Imager and Spectrograph (PIRIS) at the Mt. Wilson 100$^{\prime\prime}$ telescope. Two nearby stars were observed, $\epsilon$ Eridani and $\mu$ Her A. A faint companion was detected around $\mu$ Her A, confirming it as a proper motion companion. Furthermore, the observed H and K magnitudes of the companion were used to constrain its nature. No companions or faint structure were observed for $\epsilon$ Eridani. We found that our observations with the GAPM achieved contrast levels similar to our coronographic images, without blocking light from the central star. The mask's performance also nearly reached sensitivities reported for other ground based adaptive optics coronographs and deep HST images, but did not reach theoretically predicted contrast levels. We outline ways that could improve the performance of the GAPM by an order of magnitude or more.Comment: 8 pages, 4 figures, accepted by ApJ letter

Factor H-related protein 1 neutralizes anti-factor H autoantibodies in autoimmune hemolytic uremic syndrome.

The autoimmune form of atypical hemolytic uremic syndrome (HUS) is characterized by circulating autoantibodies against the complement regulator factor H, and is often associated with deficiency of the factor H-related proteins CFHR1 and CFHR3. Here we studied whether anti-factor H autoantibodies crossreact with CFHR1, and determined functional consequences of this. In ELISA, anti-factor H immunoglobulin G (IgG) autoantibodies from 24 atypical HUS patients bound to the short consensus repeat 20 domain of factor H, 21 antibodies also recognized CFHR1, but none CFHR3. Three patients also had anti-factor H IgA autoantibodies crossreacting with CFHR1. Analysis of the IgG fractions in CFHR1-deficient patients found that CFHR1-IgG complexes were formed during plasma exchange treatment, indicating that autoantibodies recognize CFHR1 in vivo. Recombinant CFHR1 prevented hemolysis of sheep erythrocytes caused by patient plasma containing anti-factor H IgG, but it did not inhibit red cell lysis caused by a factor H mutation (W1183 L) in the short consensus repeat 20 domain. Thus, exogenous CFHR1 provided during plasma exchange therapy may neutralize anti-factor H autoantibodies and help in the treatment of autoimmune atypical HUS

HST/STIS Ultraviolet Imaging of Polar Aurora on Ganymede

We report new observations of the spectrum of Ganymede in the spectral range 1160 - 1720 A made with the Space Telescope Imaging Spectrograph (STIS) on HST on 1998 October 30. The observations were undertaken to locate the regions of the atomic oxygen emissions at 1304 and 1356 A, previously observed with the GHRS on HST, that Hall et al. (1998) claimed indicated the presence of polar aurorae on Ganymede. The use of the 2" wide STIS slit, slightly wider than the disk diameter of Ganymede, produced objective spectra with images of the two oxygen emissions clearly separated. The OI emissions appear in both hemispheres, at latitudes above 40 degrees, in accordance with recent Galileo magnetometer data that indicate the presence of an intrinsic magnetic field such that Jovian magnetic field lines are linked to the surface of Ganymede only at high latitudes. Both the brightness and relative north-south intensity of the emissions varied considerably over the four contiguous orbits (5.5 hours) of observation, presumably due to the changing Jovian plasma environment at Ganymede. However, the observed longitudinal non-uniformity in the emission brightness at high latitudes, particularly in the southern hemisphere, and the lack of pronounced limb brightening near the poles are difficult to understand with current models. In addition to observed solar HI Lyman-alpha reflected from the disk, extended Lyman-alpha emission resonantly scattered from a hydrogen exosphere is detected out to beyond two Ganymede radii from the limb, and its brightness is consistent with the Galileo UVS measurements of Barth et al. (1997).Comment: 7 pages, 4 figures, accepted for publication in ApJ, June 1, 200

Searching for Planets in the Hyades. I. The Keck Radial Velocity Survey

We describe a high-precision radial velocity search for jovian-mass companions to main sequence stars in the Hyades star cluster. The Hyades provides an extremely well controlled sample of stars of the same age, the same metallicity, and a common birth and early dynamical environment. This sample allows us to explore the dependence of the process of planet formation on only a single independent variable: the stellar mass. In this paper we describe the survey and summarize results for the first five years.Comment: 8 pages, 3 figures; To appear in the July 2002 issue of The Astronomical Journa

A Study of the B-V Colour Temperature Relation

We attempt to construct a B-V colour temperature relation for stars in the least model dependent way employing the best modern data. The fit we obtained with the form Teff = Teff((B-V)0,[Fe/H],log g) is well constrained and a number of tests show the consistency of the procedures for the fit. Our relation covers from F0 to K5 stars with metallicity [Fe/H] = -1.5 to +0.3 for both dwarfs and giants. The residual of the fit is 66 K, which is consistent with what are expected from the quality of the present data. Metallicity and surface gravity effects are well separated from the colour dependence. Dwarfs and giants match well in a single family of fit, differing only in log g. The fit also detects the Galactic extinction correction for nearby stars with the amount E(B-V) = 0.26 +/-0.03 mag/kpc. Taking the newly obtained relation as a reference we examine a number of B-V colour temperature relations and atmosphere models available in the literature. We show the presence of a systematic error in the colour temperature relation from synthetic calculations of model atmospheres; the systematic error across K0 to K5 dwarfs is 0.04-0.05 mag in B-V, which means 0.25-0.3 mag in Mv for the K star range. We also argue for the error in the temperature scale used in currently popular stellar population synthesis models; synthetic colours from these models are somewhat too blue for aged elliptical galaxies. We derive the colour index of the sun (B-V)sun = 0.627 +/-0.018, and discuss that redder colours (e.g., 0.66-0.67) often quoted in the literature are incompatible with the colour-temperature relation.Comment: AASLaTeX (aaspp4.sty),36 pages (13 figures included), submitted to Astronomical Journal, replaced (typo in author name

Pluto's lower atmosphere structure and methane abundance from high-resolution spectroscopy and stellar occultations

Context: Pluto possesses a thin atmosphere, primarily composed of nitrogen, in which the detection of methane has been reported. Aims: The goal is to constrain essential but so far unknown parameters of Pluto's atmosphere such as the surface pressure, lower atmosphere thermal stucture, and methane mixing ratio. Methods: We use high-resolution spectroscopic observations of gaseous methane, and a novel analysis of occultation light-curves. Results: We show that (i) Pluto's surface pressure is currently in the 6.5-24 microbar range (ii) the methane mixing ratio is 0.5+/-0.1 %, adequate to explain Pluto's inverted thermal structure and ~100 K upper atmosphere temperature (iii) a troposphere is not required by our data, but if present, it has a depth of at most 17 km, i.e. less than one pressure scale height; in this case methane is supersaturated in most of it. The atmospheric and bulk surface abundance of methane are strikingly similar, a possible consequence of the presence of a CH4-rich top surface layer.Comment: AA vers. 6.1, LaTeX class for Astronomy & Astrophysics, 9 pages with 5 figures Astronomy and Astrophysics Letters, in pres