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  • 1
    ISSN: 1432-1076
    Keywords: Sphingolipid activator protein deficiency ; Farber disease ; Gaucher disease ; Krabbe disease ; Prenatal diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a patient who presented shortly after birth with hyperkinetic behaviour, myoclonia, respiratory insufficiency and hepatosplenomegaly. Gaucher-like storage cells were found in bone marrow. A liver biopsy showed massive lysosomal storage morphologically different to that in known lipid storage disorders. Biochemically, the patient had partial deficiencies of β-galactocerebrosidase, β-glucocerebrosidase and ceramidase in skin fibroblast extracts, but the sphingomyelinase activity was normal. Glucosyl ceramide and ceramide were elevated in liver tissue. Loading of cultured fibroblasts with radioactive sphingolipid precursors indicated a profound defect in ceramide catabolism. Immunological studies in fibroblasts showed a total absence of cross-reacting material to sphingolipid activator protein 2 (SAP-2). The patient died at 16 weeks of age. The fetus from his mother's next pregnancy was similarly affected. The possibility that the disorder results from a primary defect at the level of SAP-2 is discussed. We have named this unique disorder SAP deficiency.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: Monolythic arrays of 12 pn-charge coupled devices 3×1 cm2 each, have been developed and produced for the focal plane instrumentation of the high throughput European Photon Imaging Camera on XMM and the German ABRIXAS x-ray satellite mission. The design parameters have been optimized to match the properties of the x-ray imaging optics as well as the x-ray intensity, energy bandwidth, and characteristic time constants of objects to be observed. The pixel size is 150×150 μm2; read-out is performed in parallel with 64 channels in each subunit, resulting in a total of 768 channels; low noise, spectroscopic performance is realized by on-chip integrated JFET electronics; high ohmic, ultrapure bulk material allows full depletion and enhances the efficiency for higher energy x-ray detection. The fabrication process, the layout topology, and the operating conditions guarantee ten years operation in space without performance degradation. © 1997 American Institute of Physics.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1365-3040
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: Mature trees have already experienced substantial increases in CO2 concentrations during their lifetimes, and will experience continuing increases in the future. Small open-top chambers were used to enclose branchlets that were at a height of between 20 and 25 m in the canopy of the tree species Luehea seemannii Tr. & Planch. in a tropical forest in Panamá. Elevated concentrations of CO2 increased the rate of photosynthetic carbon fixation and decreased stomatal conductance of leaves, but did not influence the growth of leaf area per chamber, the production of flower buds and fruit nor the concentration of nonstructural carbohydrates within leaves. The production of flower buds was highly correlated with the leaf area produced in the second flush of leaves, indicating that the branchlets of mature trees of Luehea seemannii are autonomous to a considerable extent. Elevated levels of CO2 did increase the concentration of nonstructural carbohydrates in woody stem tissue. Elevated CO2 concentration also they increased the ratio of leaf area to total biomass of branchlets, and tended to reduce individual fruit weight. These data suggest that the biomass allocation patterns of mature trees may change under future elevated levels of CO2. Although there were no effects on growth during the experiment, the possibility of increased growth in the season following CO2 enrichment due to increased carbohydrate concentrations in woody tissue cannot be excluded.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-3040
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: The genus Ananas has its centre of origin in northern South America. In this area, several varieties of Ananas comosus are widely cultivated, and a number of wild species are found growing under variable conditions of light intensity, soil fertility and water availability. Here we report detailed daily courses of titratable acidity, and malate, citrate and free-sugars content of several cultivated varieties of A. comosus and of A. ananassoides, a closely related species growing on granitic rock-outcrops in southern Venezuela. Day-night oscillations of both malate and citrate were detected in plants growing under full sun, but malate was by far the most important organic anion associated with CAM performance in ail populations sampled. Fructose was the dominant compound in the neutral fraction, but only sucrose showed a consistent inverse relation with the cycle of titratable acidity. The diel oscillations of free sugars measured were not always enough to account for the amount of organic anions accumulated during the night. Plants cultivated under shady conditions always showed a lower night-time increase in titratable acidity and organic acids, and also smaller oscillations in the amount of free sugars than sun exposed plants. In all populations growing under full sun, osmolality increased during the night, but it was not always possible to explain these changes on the basis of variations in molar concentrations of organic acids and sugars. Besides, no diel variations in the cations K+, Ca2+ and Mg2+ were detected. K+ was always the dominant cation (K/Ca ratios ∼ 19), while Mg2+ was always higher than Ca2+ (Mg/Ca ∼ 2).
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Plant Science Letters 32 (1983), S. 115-123 
    ISSN: 0304-4211
    Keywords: Excised wheat roots ; Ion uptake ; Malate decarboxylation ; Malate synthesis
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Chromatography A 559 (1991), S. 499-504 
    ISSN: 0021-9673
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Phytochemistry 29 (1990), S. 1785-1786 
    ISSN: 0031-9422
    Keywords: 1d-1-O-methyl-muco-inositol. ; Rhizophoraceae ; Viscaceae ; Viscum album
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Plant, cell & environment 22 (1999), S. 0 
    ISSN: 1365-3040
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: The foliar content of nitrogen and the relative abundances of 13C and 15N were analysed in vascular epiphytes collected from six sites along an altitudinal gradient from tropical dry forests to humid montane forests in eastern Mexico. The proportion of epiphyte species showing crassulacean acid metabolism (CAM) (atmospheric bromeliads, thick-leaved orchids, Cactaceae, and Crassulaceae) decreased with increasing elevation and precipitation from 58 to 6%. Atmospheric bromeliads, almost all of which had δ13C values indicating CAM, were more depleted in 15N (x=−10·9‰± 2·11) than the C3 bromeliads which form water-storing tanks (−6·05‰± 2·26). As there was no difference in δ15N values between C3 and CAM orchids, the difference in bromeliads was not related to photosynthetic pathways but to different nitrogen sources. While epiphytes with strong 15N depletion appear to obtain their nitrogen mainly from direct atmospheric deposition, others have access to nitrogen in intercepted water and from organic matter decomposing on branches and in their phytotelmata. Bromeliads and succulent orchids had a lower foliar nitrogen content than thin-leaved orchids, ferns and Piperaceae. Ground-rooted hemi-epiphytes exhibited the highest nitrogen contents and δ15N values.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1600-5775
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Geosciences , Physics
    Notes: For the European X-ray multi-mirror (XMM) satellite mission and the German X-ray satellite ABRIXAS, fully depleted pn-CCDs have been fabricated, enabling high-speed low-noise position-resolving X-ray spectroscopy. The detector was designed and fabricated with a homogeneously sensitive area of 36 cm2. At 150 K it has a noise of 4 e− r.m.s., with a readout time of the total focal plane array of 4 ms. The maximum count rate for single-photon counting was 105 counts s−1 under flat-field conditions. In the integration mode more than 109 counts s−1 can be detected at 6 keV. Its position resolution is of the order of 100 µm. The quantum efficiency is higher than 90% from carbon K X-rays (277 eV) up to 10 keV. New cylindrical silicon drift detectors have been designed, fabricated and tested. They comprise an integrated on-chip amplifier system with continuous reset, on-chip voltage divider, electron accumulation layer stabilizer, large area, homogeneous radiation entrance window and a drain for surface-generated leakage current. At count rates as high as 2 × 106 counts cm−2 s−1, they still show excellent spectroscopic behaviour at room-temperature operation in single-photon detection mode. The energy resolution at room temperature is 220 eV at 6 keV X-ray energy and 140 eV at 253 K, being achieved with Peltier coolers. These systems were operated at synchrotron light sources (ESRF, HASYLAB and NLS) as X-ray fluorescence spectrometers in scanning electron microscopes and as ultra low noise photodiodes. The operation of a multi-channel silicon drift detector system is already foreseen at synchrotron light sources for X-ray holography experiments. All systems are fabricated in planar technology having the detector and amplifiers monolithically integrated on high-resistivity silicon.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European food research and technology 7 (1904), S. 6-12 
    ISSN: 1438-2385
    Source: Springer Online Journal Archives 1860-2000
    Topics: Process Engineering, Biotechnology, Nutrition Technology
    Type of Medium: Electronic Resource
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