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  • 1
    ISSN: 1432-0428
    Keywords: Congenital diabetes mellitus ; absence of beta cells ; methylmalonic acidaemia ; mutase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report on a female neonate with diabetes mellitus and methylmalonic acidaemia, who died at age 16 days. Using immunocytochemistry, electron microscopy and in situ hybridisation, we were unable to demonstrate any insulin cells in the pancreatic islets. Methylmalonic acidaemia was caused by a methylmalonyl coenzyme A mutase apoenzyme defect. The metabolic crisis of the methylmalonic acidaemia aggravated the diabetes and may explain the failure of insulin therapy. Our results suggest that the infant suffered from a congenital absence of beta cells associated with a genetically transmitted mutase apoenzyme defect.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 89 (1988), S. 4726-4736 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: The core-level photoexcitation and photoionization of SF6 were studied in the vicinity of the resonances below and above the S 2p threshold. The decay channels of the S 2p→6a1g discrete excitation were characterized, with decay leading mostly to valence-shell satellites. The S 2p continuum data show an oscillatory asymmetry parameter β(S 2p) near threshold that is virtually identical to β(Si 2p) in SiF4. It also resembles—but differs from—theoretical curves for β(S 2p) in atomic sulfur and in SF6. Data at the feature assigned as an eg shape resonance indicate strong multielectron properties for this state, because a resonance in the S 2p satellite is observed at the same photon energy as the main-line resonance. We propose a unified model which generally includes configuration interaction both in the continuum-state manifold and between discrete doubly excited states and the continua, to explain this unexpected satellite behavior. Finally, the S(L2,3VV) Auger electron asymmetry parameter shows no significant deviation from zero near the t2g and eg shape resonances.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 90 (1989), S. 3004-3009 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: Resonant photoemission has been studied above the carbon 1s ionization thresholds in gas-phase benzene and ethylene. The experimental data for both molecules include relative partial cross section and asymmetry-parameter measurements for the C 1s main line and asymmetry-parameter measurements for one C 1s shake-up satellite in each system. Resonances above the C K edge have been analyzed on the basis of their decay to either the C 1s main line or valence-hole states, and have been tentatively assigned as either shape resonances or doubly excited states according to their observed one-electron or many-electron decay, respectively. The importance of determining the resonant behavior of all available photoemission channels in the proximity of a resonance is thus illustrated.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 89 (1988), S. 6096-6102 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: Zero kinetic energy (ZKE) spectra of N2, CO, C2H4, and C6H6 were taken across the N1s (N2) and C1s ionization thresholds. Discrete resonances at subthreshold photon energies were observed and were found to become more intense as threshold is approached relative to the same peaks in absorption spectra. For N2 the satellite/main line branching ratios at threshold are: 11(1)% for the 419.7(1) eV binding energy satellite, and 2.3(1.0)% for the 426.5(1) eV binding energy satellite. For CO, the branching ratio for the 304.6(1) eV binding energy satellite is 15(2)% at its threshold. Branching ratios at threshold are also determined for the satellites of C6H6 and C2H4. Decay characteristics and assignments of the continuum features of C6H6 and C2H4 are also discussed.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Centaurus 21 (1977), S. 0 
    ISSN: 1600-0498
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine , Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Centaurus 18 (1974), S. 0 
    ISSN: 1600-0498
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine , Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Centaurus 12 (1968), S. 0 
    ISSN: 1600-0498
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine , Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 106 (1997), S. 8931-8934 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: We have improved a newly developed experimental scheme for high resolution pulsed field ionization photoelectron (PFI-PE) studies [Hsu et al., Rev. Sci. Instrum. (in press)] using the high resolution monochromatized multibunch undulator synchrotron source of the Chemical Dynamics Beamline at the Advanced Light Source. This improved scheme makes possible PFI-PE measurements with essentially no contamination by background electrons arising from direct photoionization and prompt autoionization processes. We present here a preliminary analysis of the rotationally resolved PFI-PE spectrum for O2 obtained at a resolution of 0.5 meV (full-width-at-half-maximum) in the photon energy range of 18.1–19.4 eV, yielding accurate ionization energies for the transitions O2+(b 4Σg−, v+=0–9, N+=1)←O2(X 3Σg−, v=0, N=1). © 1997 American Institute of Physics.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    College Park, Md. : American Institute of Physics (AIP)
    The Journal of Chemical Physics 105 (1996), S. 3950-3961 
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: Using the high resolution vacuum ultraviolet (vuv) photon source provided by the monochromatized undulator synchrotron radiation of the Chemical Dynamics Beamline at the Advanced Light Source, we have measured the photoionization efficiency (PIE) spectrum for Ne in the energy range of 21.56–21.67 eV at a wavelength resolution of 0.3 meV [full width at half-maximum (FWHM)]. The PIE spectra for Ne obtained using 0.76 and 2.4 V/cm electric fields reveal autoionizing features attributable to the Rydberg states Ne[2p5ns′(1/2)1; n=14–29] and Ne[2p5nd′(3/2)1; n=12–35] converging to the spin–orbit excited Ne+(2P1/2) state. The positions of these Rydberg states are compared to previous experimental results and those calculated using the quantum defects and IE for Ne+(2P1/2) given in Moore [Natl. Stand Ref. Data Ser. Natl. Bur. Stand. 35 (1971)]. We have also observed mass analyzed threshold ions (MATI) for Ne formed in the Ne+(2P3/2,1/2) states. For Ar, only the MATI peak for Ar+(2P3/2) is observed. The failure to observe the MATI peak for Ar+(2P1/2) is attributed to shorter lifetimes of high-n Ar[3p5ns′(1/2)1] and Ar[3p5nd′(3/2)1] Rydberg states compared to the minimum time required for separating the prompt Ar+ ions from the field ionized Ar+ ions in this experiment. The MATI peaks for Ne+(2P3/2,1/2) achieves a resolution of 0.7 meV (FWHM). These MATI spectra for Ne and Ar reported here represent the first such studies made using a cw vuv light source and dc electric fields. © 1996 American Institute of Physics.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1089-7690
    Source: AIP Digital Archive
    Topics: Physics , Chemistry and Pharmacology
    Notes: For condensed benzene ice layers, core photoabsorption near edge structure (x-ray absorption; recorded by Auger electron yield measurements), decay electron spectra for resonant and nonresonant excitation, and fragmentation as evident in yields of hydrogen and other ions, have been measured in the C1s region. The absorption spectrum is better resolved than most previously published spectra, exhibits some new features, and shows a high degree of parallelity to the spectrum of isolated molecules. Interestingly, the hydrogen ion yield indicates a particular dissociativeness of a certain core excitation resonance, X, which in the molecule has previously been assigned to a Rydberg state. This selective dissociation suggests that the responsible excitation is strongly antibonding for the carbon–hydrogen bond, while the degenerate Rydberg states broaden into a conduction band in the solid; and that the bond breaking probably occurs or at least starts in the core-excited state, thus proceeding on an extremely short time scale, similarly to observations for other hydrogen-containing molecules. The decay spectra are analyzed in terms of autoionization vs normal Auger decay and indicate that, apart from the first strong π resonance (which leads to pure autoionization) and the X resonance, the core resonances partly or fully ionize before core decay takes place. For the X resonance, the decay spectrum contains a contribution which cannot be assigned to intact benzene; this is taken as additional evidence for ultrafast dissociation, i.e., competitive with core decay. We use these results for a discussion of the influence of condensation on excitation, decay, and fragmentation.
    Type of Medium: Electronic Resource
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