C9orf72-mediated ALS and FTD: multiple pathways to disease

The discovery that repeat expansions in the C9orf72 gene are a frequent cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has revolutionized our understanding of these diseases. Substantial headway has been made in characterizing C9orf72-mediated disease and unravelling its underlying aetiopathogenesis. Three main disease mechanisms have been proposed: loss of function of the C9orf72 protein and toxic gain of function from C9orf72 repeat RNA or from dipeptide repeat proteins produced by repeat-associated non-ATG translation. Several downstream processes across a range of cellular functions have also been implicated. In this article, we review the pathological and mechanistic features of C9orf72-associated FTD and ALS (collectively termed C9FTD/ALS), the model systems used to study these conditions, and the probable initiators of downstream disease mechanisms. We suggest that a combination of upstream mechanisms involving both loss and gain of function and downstream cellular pathways involving both cell-autonomous and non-cell-autonomous effects contributes to disease progression

Inverse Compton X-ray halos around high-z radio galaxies: A feedback mechanism powered by far-infrared starbursts or the CMB?

We report the detection of extended X-ray emission around two powerful high-z radio galaxies (HzRGs) at z~3.6 (4C03.24 and 4C19.71) and use these to investigate the origin of extended, Inverse Compton (IC) powered X-ray halos at high z. The halos have X-ray luminosities of Lx~3e44 erg/s and sizes of ~60kpc. Their morphologies are broadly similar to the ~60-kpc long radio lobes around these galaxies suggesting they are formed from IC scattering by relativistic electrons in the radio lobes, of either CMB or FIR photons from the dust-obscured starbursts in these galaxies. These observations double the number of z>3 HzRGs with X-ray detected IC halos. We compare the IC X-ray to radio luminosity ratios for these new detections to the two previously detected z~3.8 HzRGs. Given the similar redshifts, we would expect comparable X-ray IC luminosities if CMB mm photons are the seed field for the IC emission. Instead the two z~3.6 HzRGs, which are ~4x fainter in the FIR, also have ~4x fainter X-ray IC emission. Including a further six z>2 radio sources with IC X-ray halos from the literature, we suggest that in the more compact (lobe sizes <100-200kpc), majority of radio sources, the bulk of the IC emission may be driven by scattering of locally produced FIR photons from luminous, dust-obscured starbursts within these galaxies, rather than CMB photons. The resulting X-ray emission can ionise the gas on ~100-200-kpc scales around these systems and thus form their extended Ly-alpha emission line halos. The starburst and AGN activity in these galaxies are thus combining to produce an effective and wide-spread "feedback" process, acting on the long-term gas reservoir for the galaxy. If episodic radio activity and co-eval starbursts are common in massive, high-z galaxies, then this IC-feedback mechanism may affect the star-formation histories of massive galaxies. [Abridged

Energy feedback from x-ray binaries in the early universe

X-ray photons, because of their long mean-free paths, can easily escape the galactic environments where they are produced, and interact at long distances with the intergalactic medium, potentially having a significant contribution to the heating and reionization of the early universe. The two most important sources of X-ray photons in the universe are active galactic nuclei (AGNs) and X-ray binaries (XRBs). In this Letter we use results from detailed, large scale population synthesis simulations to study the energy feedback of XRBs, from the first galaxies (z ∼ 20) until today. We estimate that X-ray emission from XRBs dominates over AGN at z ≳ 6-8. The shape of the spectral energy distribution of the emission from XRBs shows little change with redshift, in contrast to its normalization which evolves by ∼4 orders of magnitude, primarily due to the evolution of the cosmic star-formation rate. However, the metallicity and the mean stellar age of a given XRB population affect significantly its X-ray output. Specifically, the X-ray luminosity from high-mass XRBs per unit of star-formation rate varies an order of magnitude going from solar metallicity to less than 10% solar, and the X-ray luminosity from low-mass XRBs per unit of stellar mass peaks at an age of ∼300 Myr and then decreases gradually at later times, showing little variation for mean stellar ages ≳ 3 Gyr. Finally, we provide analytical and tabulated prescriptions for the energy output of XRBs, that can be directly incorporated in cosmological simulations. © 2013. The American Astronomical Society. All rights reserved

The X-ray-to-optical properties of optically selected active galaxiesover wide luminosity and redshift ranges

We present partial-correlation analyses that examine the strengths of the relationships between L_UV, L_X, Alpha_OX, and redshift for optically-selected AGNs. We extend the work of Strateva et al. (2005), that analyzed optically-selected AGNs from the Sloan Digital Sky Survey (SDSS), by including 52 moderate-luminosity, optically-selected AGNs from the COMBO-17 survey with corresponding deep (~250 ks to 1 Ms) X-ray observations from the Extended Chandra Deep Field-South. The COMBO-17 survey extends ~3 magnitudes deeper than the SDSS and probes the moderate-luminosity AGNs that numerically dominate the AGN population in the Universe. We also include recently published observations of 19 high-redshift, optically-selected AGNs, and 46 luminous, low-redshift AGNs from the Bright Quasar Survey. The full sample used in our analysis consists of 333 AGNs, extending out to z~6, with 293 (88%) having X-ray detections. The sample spans five decades in UV luminosity and four decades in X-ray luminosity. We confirm that Alpha_OX is strongly anti-correlated with L_UV (13.6 sigma), the highest significance found for this relation to date, and find evidence suggesting that the slope of this relation may be dependent on L_UV. We find that no significant correlation exists between Alpha_OX and redshift (1.3 sigma), and constrain the maximum evolution of AGN UV-to-X-ray flux ratios to be less than 30% (1 sigma) out to z=5. Using our sample's high X-ray detection fraction, we also find a significant anti-correlation (3.0 sigma) between Alpha_OX and L_X . We make comparisons to earlier studies on this topic and discuss implications for X-ray vs. optical luminosity functions.Comment: 16 pages, 10 figures, emulateapj. Accepted for publication in A

Young Black Hole and Neutron Star Systems in the Nearby Star-forming Galaxy M33: The NuSTAR View

Abstract We can learn about the formation and evolution of compact objects, such as neutron stars and black holes (BHs), by studying the X-ray emission from accreting systems in nearby star-forming galaxies. The hard (E &gt; 10 keV) X-ray emission in particular allows strong discrimination among the accretion states and compact object types. We conducted a NuSTAR survey (∼600 ks) of the Local Group spiral galaxy M33 to study the distribution of X-ray binary (XRB) accretors in an actively star-forming environment. We constructed color–intensity and color–color diagrams to infer XRB accretion states. Using these diagrams, we have classified 28 X-ray sources in M33 by comparing their hard X-ray colors to those of known systems. Four sources lie in the parameter space occupied by X-ray pulsars, while 8, 10, and 4 sources lie in the parameter space occupied by BHs in the hard, intermediate, and soft states, respectively. The known ultraluminous X-ray source M33 X-8 is also found to be consistent with that source type. Some sources overlap within the Z/Atoll sources due to the overlap of the two categories of BHs and Z/Atoll sources. In contrast to a similar NuSTAR survey of M31 (with a low-mass XRB-dominant population), the source population in M33 is dominated by high-mass XRBs (HMXBs), allowing the study of a very different population with similar sensitivity due to the galaxy's similar distance. This characterization of a population of HMXB accretion states will provide valuable constraints for theoretical XRB population synthesis studies to their formation and evolution.</jats:p

On the Nature of AGN and Star Formation Enhancement in the <em>z</em> = 3.1 SSA22 Protocluster: The HST WFC3 IR View

\ua9 2021 Institute of Physics Publishing. All rights reserved. We examine possible environmental sources of the enhanced star formation and active galactic nucleus (AGN) activity in the z = 3.09 SSA22 protocluster using Hubble WFC3 F160W (~1.6 \ub5m) observations of the SSA22 field, including new observations centered on eight X-ray selected protocluster AGN. To investigate the role of mergers in the observed AGN and star formation enhancement, we apply both quantitative (S\ue9rsic-fit and Gini-M20) and visual morphological classifications to F160W images of protocluster Lyman-break galaxies (LBGs) in the fields of the X-ray AGN and z ~ 3 field LBGs in SSA22 and GOODS-N. We find no statistically significant differences between the morphologies and merger fractions of protocluster and field LBGs, though we are limited by small number statistics in the protocluster. We also fit the UV-to-near-IR spectral energy distributions of F160W-detected protocluster and field LBGs to characterize their stellar masses and star formation histories. We find that the mean protocluster LBG is a factor of ~2 times more massive and more attenuated than the mean z ~ 3 field LBG. We take our results to suggest that ongoing mergers are not more common among protocluster LBGs than field LBGs, though protocluster LBGs appear to be more massive. We speculate that the larger mass of the protocluster LBGs contributes to the enhancement of SMBH mass and accretion rate in the protocluster, which in turn drives the observed protocluster AGN enhancement

The population of BzK-selected ulirgs at z similar to 2

We investigate the multi-wavelength emission of BzK selected star forming galaxies at z~2 in the Great Observatories Origins Deep Survey (GOODS) North region. Most (82%) of the sources are individually detected at 24um in the Spitzer MIPS imaging, and one fourth (26%) in the VLA radio data. Significant detections of the individually undetected objects are obtained through stacking in the radio, submm and X-ray domains. The typical star forming galaxy with stellar mass ~10^{11}Mo at z=2 is an Ultra-luminous Infrared Galaxy (ULIRG), with L_IR ~ 1-2x10^{12}Lo and star formation rate SFR 200-300Mo/yr, implying a comoving density of ULIRGs at z=2 at least 3 orders of magnitude above the local one. SFRs derived from the reddening corrected UV luminosities agree well, on average, with the longer wavelength estimates. The high 24um detection rate suggests a relatively large duty cycle for the BzK star forming phase, consistently with the available independent measurements of the space density of passively evolving galaxies at z>1.4. If the IMF at z=2 is similar to the local one, and in particular is not a top-heavy IMF, this suggests that a substantial fraction of the high mass tail (>10^{11}Mo) of the galaxy stellar mass function was completed by z~1.4.Comment: 4 pages, 2 figures, ApJ Letters in pres