Friedreichs ataxia (FRDA) is a progressive spinocerebellar ataxia (SCA) inherited as an autosomal recessive trait. The neurodegeneration, cardiomyopathy, diabetes mellitus and skeletal deformities characteristic to FRDA result from a deficiency in the mitochondrial protein frataxin. Frataxin chaperones iron to heme and iron-sulfur clusters and its deficiency causes mitochondrial iron accumulation and oxidative stress.<p> To address the effect of frataxin deficiency on mitochondrial iron chemistry, mitochondria were isolated from FRDA and control fibroblasts. X-ray absorption spectroscopy showed that ferrihydrite was the predominant form of iron in both. Near edge analysis showed that the ferrihydrite in the FRDA mitochondria resembled the highly organized ferrihydrite of ferritin. Western blotting confirmed that FRDA mitochondria had 3-fold more holoferritin containing stainable iron. I conclude that mitochondria from FRDA fibroblasts mineralize excess iron as ferrihydrite within mitochondrial ferritin.<p> To address how cellular iron dysregulation affected metal distribution in brain and spinal cord, a new synchrotron imaging technique, rapid-scanning x-ray fluorescence (RS-XRF) was employed and validated. Brain structures were readily identified by their unique metal content and distribution. This showed that RS-XRF could be used to reveal metal pathologies associated with diseases of metal metabolism such as FRDA. Since human FRDA tissues were not available for a detailed study, RS-XRF was employed to study the distribution of metals in normal cerebellum, a major site of FRDA-associated neurodegeneration, and to localize and quantify metals in the brain and spinal cord from a patient with a SCA of unknown aetiology. The motivation for this work is the prospect of future systematic studies on metal pathology in neurodegenerative diseases with direct application to FRDA. Novel findings arising from this work were the metal segmentation of the dentate nucleus, the high copper content of the olivary region and the different metal content of lesions at different stages of neurodegeneration. My results suggest that not only iron, but also copper and zinc may play a role in the physiopathology of neurodegeneration. Therefore, all three metals should be investigated in FRDA and other SCA of both known and unknown aetiologies to identify possible new therapeutic targets.

Soil organic N (SON) comprises 90% of all N in surface soils, yet as much as half remains in forms which are chemically unknown or, at best, poorly understood. Analytical methods such has pyrolysis field-ionization mass spectrometry (Py-FIMS) and 15N cross polarization magic-angle spinning nuclear magnetic resonance (CPMAS-NMR) spectroscopy are widely used for the characterization of SON; however, these methods have limitations which contribute to the gaps in our understanding of SON chemistry. For example, Py-FIMS may produce heat-induced secondary compounds, and 15N-NMR may lack sensitivity and resolution for experiments at natural 15N abundance. X-ray absorption near edge structure (XANES) spectroscopy probes the bonding environment of individual elements. The application of this technique to complex environmental samples such as soil is still in its infancy, but early studies suggest that this technique may help resolve SON molecular structure. This dissertation sought to develop and apply synchrotron-based N and C K-edge XANES spectroscopy to the study of soil and soil extracts to determine the structures in which SON is bound. In these studies, Py-FIMS was coupled with XANES as a corroboratory technique.<p> Initial methodological development resulted in a calibration method whereby N2 gas generated in ammonium-containing salts was used to calibrate a soft X-ray beamline at the N K-edge. Although XANES can produce secondary compound artifacts, contrary to early assertions that it is a non-destructive technique, it was shown in a second study that beam-induced decomposition can be minimized by moving the beam to a fresh spot between scans.<p> Three applied studies exploring SON composition were conducted. These studies followed a spatial gradient ranging from the landscape scale, through a rhizosphere study, and ended with a study of glomalin-related soil protein (GRSP). Glomalin-related soil protein is a persistent soil glycoprotein of arbuscular mycorrhizal origin (AMF) implicated in aggregation and long-term C and N storage. Nitrogen and C K-edge XANES and Py-FIMS were used in all studies, and GRSP was further characterized using proteomics techniques.<p> Soil organic N composition was largely controlled by topographic position, and to a lesser degree, by cultivation. Divergent (i.e., water shedding) positions were enriched in carbohydrates and low molecular weight lignins, whereas convergent, depressional and level positions showed enrichment in lipid-type compounds. These differences were attributed to tillage-induced redistribution of soil, and water movement from upper to lower slope positions. Nitrogen XANES revealed a unique form of organic N, identified as N-bonded aromatics, particularly in the divergent positions.<p> Rhizosphere soil was enriched in higher molecular weight lipid-type materials and depleted in low molecular weight polar compounds. This was attributed to increased input of fresh plant material and higher microbial turnover in the rhizosphere. Nitrogen-bonded aromatics also were detected in the rhizosphere.<p> The GRSP extracts were characterized as mostly proteinaceous, but also contained many co-extracted, non-protein compounds. Despite being previously described as a glycoprotein, only weak carbohydrate signals were observed. Proteomics-based assessment of GRSP showed no homology to any proteins of AMF origin, instead showing homology with thioredoxin and with heat-stable soil proteins. This may be because protein databases do not yet contain glomalin-related sequences, or that glomalin is homologous to non-AMF soil proteins.<p> This dissertation demonstrated that N XANES is a sensitive and novel method for characterizing SON, and can be used complementarily with other analytical techniques such as Py-FIMS and proteomics. The continued development of XANES will provide a useful tool for SOM research into the future.

<p>The High Intensity Gamma-ray Source (HIGS) at Duke University is a world class Compton light source facility. At the HIGS, a Free-Electron Laser (FEL) beam is Compton scattered with an electron beam in the Duke storage ring to produce an intense, highly polarized, and nearly monoenergetic gamma-ray beam with a tunable energy from about 1 MeV to 100 MeV. This unique gamma-ray beam has been used in a wide range of basic and application research fields from nuclear physics to astrophysics, from medical research to homeland security and industrial applications.</p><p>The capability of accurately predicting the spatial, spectral and temporal characteristics of a Compton gamma-ray beam is crucial for the optimization of the operation of a Compton light source as well as for the applications utilizing the Compton beam. In this dissertation, we have successfully developed two approaches, an analytical calculation method and a Monte Carlo simulation technique, to study the Compton scattering process. Using these two approaches, we have characterized the HIGS beams with varying electron beam parameters as well as different collimation conditions. Based upon the Monte Carlo simulation, an end-to-end spectrum reconstruction method has been developed to analyze the measured energy spectrum of a HIGS beam. With this end-to-end method, the underlying energy distribution of the HIGS beam can be uncovered with a high degree of accuracy using its measured spectrum. To measure the transverse profile of the HIGS beam, we have developed a CCD based gamma-ray beam imaging system with a sub-mm spatial resolution and a high contrast sensitivity. This imaging system has been routinely used to align experimental apparatus with the HIGS beam for nuclear physics research. </p><p>To determine the energy distribution of the HIGS beam, it is important to know the energy distribution of the electron beam used in the collision. The electron beam energy and energy spread can be measured using the Compton scattering technique. In order to use this technique, we have developed a new fitting model directly based upon the Compton scattering cross section while taking into account the electron-beam emittance and gamma-beam collimation effects. With this model, we have successfully carried out a precise energy measurement of the electron beam in the Duke storage ring. </p><p>Alternatively, the electron beam energy can be measured using the Resonant Spin Depolarization technique, which requires a polarized electron beam. The radiative polarization of an electron beam in the Duke storage ring has been studied as part of this dissertation program. From electron-beam lifetime measurements, the equilibrium degree of polarization of the electron beam has been successfully determined. With the polarized electron beam, we will be able to apply the Resonant Spin Depolarization technique to accurately determine the electron beam energy. This on-going research is of great importance to our continued development of the HIGS facility.</p> / Dissertation

This thesis focuses on the characterization of soluble iron in ambient/urban and source emission aerosols, primarily focusing on the sources and atmospheric processes contributing to iron solubility. Multiple techniques, including bulk and single particle measurements, were used to investigate the complex chemistry of iron solubility. A technique was developed and validated (PILS-LWCC), allowing for 12-minute measurements of water-soluble ferrous iron (WS_Fe(II)) in aerosols with a limit of detection of 4.6 ng m-3 and 12% relative uncertainty. The PILS-LWCC was deployed at several urban field sites (Atlanta, GA and Dearborn, MI) and a biomass burning event to determine major sources and atmospheric processes of WS_Fe(II) in aerosols. PILS-LWCC measurements suggest that industrial and biomass burning are sources of WS_Fe(II). In addition, acid-processing mechanisms also appeared to influence WS_Fe(II) concentrations, based on a strong correlation between WS_Fe(II) and SO42- (r2 = 0.76) as well as apparent aerosol acidity (r2 =0.78) during a field campaign in Atlanta, GA. Synchrotron-based techniques, such as X-ray Absorption Near-Edge Structure (XANES) spectroscopy and micro X-ray fluorescence measurements, were also used to identify the chemical composition (redox state and phase) and mixing state (two properties that may influence iron solubility) of source emission and ambient single iron-containing particles. These single particle measurements were used in conjunction with bulk iron solubility to assess the influence of chemical composition and mixing state on iron solubility. Single particle (synchrotron-based) and bulk iron solubility measurements suggested that iron solubility is not primarily driven by chemical composition in source emission and ambient particles. Differences in iron solubility, however, were related to single particle sulfur content in ambient and source emission aerosols, suggesting that similar sources/atmospheric processes control iron solubility in these samples. The relationship between iron solubility and sulfur content corresponded well with bulk ground-based measurements of ambient aerosol using the PILS-LWCC. Combined single particle and bulk online measurements provide compelling evidence that atmospheric acid processing, involving sulfur-containing acids (H2SO4), is an important formation route of soluble iron in ambient urban aerosols. The results of this thesis provide valuable information to further understanding the controls of iron solubility in atmospheric aerosols.

No description available.

Bi-annual report on the activities at the ROssendorf BeamLine (ROBL) at the ESRF in Grenoble. The report contains selected contributions on actual research topics, a list of all scheduled experiments, and short experimental reports.

The paper describes the Rossendorf Beamline (ROBL) built by the Forschungszentrum Rossendorf at the ESRF. ROBL comprises two different and independently operating experimental stations: a radiochemistry laboratory for X-ray absorption spectroscopy of non-sealed radioactive samples and a general purpose materials research station for X-ray diffraction and reflectometry mainly of thin films and interfaces modified by ion beam techniques. The radiochemistry set-up is worldwide an unique installation at a modern synchrotron radiation source.

<p>This thesis presents experimental studies of gas phasemolecular relaxation after excitation with synchrotron photonsin the 15-35 eV and in the 70-350 eV regions.</p><p>In the 15-35 eV region, molecular relaxation by neutraldissociation processes and non</p><p>Franck-Condon effects in N2 and O2 molecules have beenstudied by means of dispersed fluorescence and photoelectronspectroscopy experimental techniques, respectively. From thedispersed fluorescence data, excitation functions for themeasured atomic fluorescence spectra have been obtained. Fromthe recorded photoelectron spectra vibrational branching ratioshave been produced. The results obtained reveal that Rydbergseries and singly and doubly excited valence states of theappropriate symmetry energetically accessible in the studiedregion and interactions between themaccount for most of theobserved effects in these two type of experiments.</p><p>In the 70-350 eV range, molecular relaxation processesresulting in fragmentation of CD4 and SF6 after absorption ofsynchrotron light have been studied by energy resolved electronion coincidence technique using a multicoincidence experimentalstation developed by our group during the last five years forsuch type of experiments. The coincidence measurements yieldedmass spectra from which information about the kinematics of thedetected fragments has been deduced by means of Monte Carlosimulations of the experimental peak shapes. The obtainedresults show completely different dissociation patternsdepending on the molecular electronic states studied. Thesepatterns reflect the bonding properties of the excited orbitalsand they permit the description and in some cases theidentification of the different molecular relaxation pathwaysobserved. The achievements presented in this thesis exemplifythe potential of the multicoincidence station used in thereported experiments.</p>

<p>A new experimental set-up for gas phase fluorescence studies using synchrotron radiation has been designed and constructed to perform simultaneously total and dispersed fluorescence measurements. </p><p>Neutral photodissociation of CO has been investigated after excitation with 19-26 eV photons. Fluorescence from 3p 3P, 3p 3S and 3p 1D excited states in carbon was recorded and interpreted by ab initio calculations. The population and dissociation of states belonging to the C and D Rydberg series in CO seem to explain the production of the observed triplet states but not the 3p 1D state. </p><p>Neutral photodissociation of NO is reported in the 17-26 eV energy range. No known molecular states can account for the collected data. New information regarding the precursor states of the observed neutral dissociation is provided by ab initio calculations. </p><p>Autoionization of superexcited states in molecular nitrogen is evidenced by strong deviations of the Franck-Condon ratio in the fluorescence of the N2+ B state. Ab initio calculations predict the existence of autoionizing-excited states that may account for some of the observed structures in the 20-46 eV energy range. </p><p>Selective molecular fluorescence from the npó1Óu+ and npð 1Ðu (n=3-7) Rydberg levels to the E,F 1Óg+ state in H2 was recorded and rotationally analyzed. Vibrational levels of the E,F 1Óg+ state (vEF =0,1,3,6-10) are determined. The predissociation of npð 1Ð+ levels is observed in agreement with the literature. </p><p>Fragmentation of SF6 was investigated after excitation with 25–80 eV photons. Dispersed fluorescence measurements reveal the emission of S, S+, F and F+ excited atoms. These fragments are produced after single, double and triple excitations as well as direct ionizations and shake-ups in SF6. </p><p>Photoabsorption and fluorescence yield have been measured in SF5CF3 using 10-30eV photons. The photoabsorption spectrum can be explained in terms of its similarities to those of the SF6 and CF4 molecules. The dispersed and un-dispersed fluorescence resemble those of the CF3X family. Several features suggest the migration of an F atom across the S-C bond that fragments the molecule producing excited CF4. </p><p>Doubly excited states of H2 have been investigated in the range of 26-60 eV by monitoring Balmer á emission. The experimental data show the already known emission correlated with the fragmentation of the Q1 and Q2 states, and new features which could be attributed to dissociative photoionization and higher lying doubly excited states Qn (n>2) of the hydrogen molecule</p>

L'objectif de ce travail a été d'étudier, à l'échelle atomique et depuis les tous premiers stades, la croissance de l'oxyde de praséodyme sur des substrats de silicium orientés (111) et (001). L'étude se focalise sur la caractérisation des propriétés structurales, chimiques, et électroniques de Pr2O3 déposé sur Si(111) et Si(001), avec un accent plus particulier sur cette dernière surface, importante sur le plan technologique. Cet oxyde de terre-rare a été considéré ces dernières années comme un bon candidat "high-k'' pour se substituer à SiO2 comme oxyde de grille dans les transistors CMOS, afin de "miniaturiser'' davantage les composants électroniques. Dans ce contexte, les propriétés des surfaces et des interfaces commencent à dominer sur les performances des dispositifs à base de silicium, puisque le rapport surface/volume augmente. Comme les basses dimensions de ces structures les rendent difficilement étudiables, la brillance de la radiation synchrotron a été employée pour des analyses de diffraction des rayons X et de spectroscopie de photoélectrons. Les résultats ont été complétés par d'autres techniques de science des surfaces, telles que la microscopie à effet tunnel, la spectroscopie Auger et la diffraction d'électrons à basse énergie. Une couche epitaxiale de Pr2O3 hexagonal a été trouvée sur Si(111). Sur la surface (001), une couche ordonnée de Pr2O3 cubique est recouverte par un silicate. Puisque les surfaces atomiquement propres sont le point de départ de la croissance, elles ont également été caractérisées. En particulier, la structure atomique de la surface reconstruite Si(001)-2x1 a été analysée.