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  • 1
    Electronic Resource
    Electronic Resource
    Typical Friedreich's ataxia without GAA expansions and GAA expansions without typical Friedreich's ataxia (2000)
    Springer
    Journal of neurology 247 (2000), S. 346-355 
    Details
    ISSN: 1432-1459
    Keywords: Key words Friedreich's ataxia ; GAA trinucleotide repeat ; Mandatory diagnostic criteria ; Intrafamilial phenotypic variability ; Sensitivity and specificity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We clinically assessed and performed polymerase chain reaction analysis for the GAA trinucleotide repeat expansion in 103 patients from 73 families in Ireland, with a prior clinical diagnosis of Friedreich's ataxia (FA) or an unclassified progressive ataxic syndrome. The patients were classified as “typical” or “atypical” FA according to Harding's mandatory clinical diagnostic criteria. All patients underwent blood glucose analysis, and electrocardiography and echocardiography was performed in 99 and 101 patients, respectively. Mutation screening for expanded CAG trinucleotide repeats, associated with spinocerebellar ataxia (SCA) 1, 2, 3 and 6 was performed in 86 patients overall, including all GAA negative patients. Forty-nine of 56 typical patients and 13 of 47 atypical patients were either homozygous or heterozygous for the GAA expansion. Seven patients with a typical FA phenotype were negative for the GAA expansion. Although one of these patients had vitamin E deficiency, and two had raised α-fetoprotein levels, three other GAA negative patients with a typical FA phenotype had no other identifiable cause for their ataxia, once again raising the possibility of locus heterogeneity in FA. It is also possible that these patients have two point mutations in the X25 gene, or that they have another ataxic syndrome mimicking the FA phenotype. Two families who were homozygous for the GAA expansion exhibited intrafamilial phenotypic variability. Only one GAA negative patient had the SCA 3 mutation, and this was the only patient in the study with a possible autosomal dominant inheritance pattern. In the homozygous GAA population typical patients had significantly more repeats on the smaller allele than atypical patients, and there was an inverse relationship between the number of repeats on the smaller allele and the age at presentation. There was also an inverse relationship between the repeat size on both the larger and the smaller of the two alleles and the age at becoming wheelchair bound. There was no significant relationship between repeat size and the other indices of disease severity, including the presence or absence of diabetes or cardiomyopathy. This is the first large study of an Irish population with progressive ataxia that has shown a similar phenotype/genotype relationship to studies of FA in other European and non-European populations. The relatively low sensitivity and specificity of Harding's clinical diagnostic criteria must be appreciated when clinically assessing patients with a progressive ataxic patients with a progressive ataxic syndrome. Although molecular genetic analysis now plays an essential role in diagnosis and classification, patients with a typical FA phenotype without any identifiable cause for their ataxia exist.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004150050601
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  • 2
    Electronic Resource
    Electronic Resource
    Outcome and quality of life after treatment with hemofiltration/hemodiafiltration (1998)
    Springer
    Intensive care medicine 24 (1998), S. 93-94 
    Details
    ISSN: 1432-1238
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001340050524
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  • 3
    Electronic Resource
    Electronic Resource
    Latent inhibition: A trace conditioning phenomenon?
    Amsterdam : Elsevier
    Learning and Motivation 18 (1987), S. 185-201 
    Details
    ISSN: 0023-9690
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Education , Psychology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1016/0023-9690(87)90010-5
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  • 4
    Electronic Resource
    Electronic Resource
    Microwave properties of highly oriented YBa2Cu3O7−x thin films (1990)
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 56 (1990), S. 1178-1180 
    Details
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: We have performed intra- and extra-cavity microwave frequency (1–100 GHz) measurements on high quality Y1Ba2Cu3O7−x superconducting thin films on (100) LaAlO3 substrates. The ∼0.3 μm thin films fabricated by the pulsed laser deposition technique exhibit superconducting transition temperatures 〉90 K, as determined by resistivity and ac susceptibility measurements, and critical current densities of 5×106 A/cm2 at 77 K. Moreover, ion beam channeling minimum yields of ∼3% were measured, indicating the extremely high crystalline quality of films grown on the LaAlO3 substrate. Microwave surface resistance values at 77 K for these films are found to be more than one to two orders of magnitude lower than for copper at 77 K for almost the entire frequency range explored. We postulate that the reason we observe such low surface resistances in these films is the virtual absence of grain and phase boundaries coupled with the high degree of crystallinity. Furthermore, we believe that the residual resistance measured below Tc is at present dominated by losses occurring in the substrate and the cavities rather than by losses intrinsic to the Y-Ba-Cu oxide superconductor.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.103331
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  • 5
    Electronic Resource
    Electronic Resource
    Physical-Biological Interactions Influencing Marine Plankton Production (1993)
    Palo Alto, Calif. : Annual Reviews
    Annual Review of Ecology, Evolution, and Systematics 24 (1993), S. 555-585 
    Details
    ISSN: 0066-4162
    Source: Annual Reviews Electronic Back Volume Collection 1932-2001ff
    Topics: Biology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1146/annurev.es.24.110193.003011
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  • 6
    Electronic Resource
    Electronic Resource
    The effect of soil type on phosphorus sorption capacity and desorption dynamics in Irish grassland soils (2001)
    Oxford, UK : Blackwell Publishing Ltd
    Soil use and management 17 (2001), S. 0 
    Details
    ISSN: 1475-2743
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Geosciences , Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Notes: Abstract. The phosphorus (P) sorption and desorption dynamics of eleven major agricultural grassland soil types in Ireland were examined using laboratory techniques, so that soils vulnerable to P loss might be identified. Desorption of P from soil using the iron-oxide paper strip test (Pfeo), water extractable P (Pw) and calcium chloride extractable P (Pcacl2) depended on soil P status in all soils. However, soil types with high organic matter levels (OM), namely peat soils (%OM 〉30), had lower Pfeo and Pw but higher Pcacl2 values compared to mineral soils at similar soil test P levels. Phosphorus sorption capacity remaining (PSCr) was measured using a single addition of P to soils and used to calculate total P sorption capacities (PSCt) and degree of P saturation (DPS). Phosphorus sorption capacities correlated negatively with % OM in soils indicating that OM may inhibit P sorption from solution to soil. High organic matter soils exhibited low P sorption capacities and poor P reserves (total P, oxalate extractable P) compared to mineral soils. Low P sorption capacities (PSCt) in peat soils were attributed to OM, which blocked or eliminated sorption sites with organic acids, therefore, P remained in the soil solution phase (Pcacl2). In this work, peat and high organic matter soils exhibited P sorption and desorption characteristics which suggest that these soils may not be suitable for heavy applications of manure or fertilizer P owing to their low capacities for P sorption and storage.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1475-2743.2001.tb00003.x
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  • 7
    Electronic Resource
    Electronic Resource
    Low-loss substrate for epitaxial growth of high-temperature superconductor thin films (1988)
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 53 (1988), S. 2677-2679 
    Details
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: A perovskite-like single-crystal substrate material has been investigated that simultaneously permits epitaxial growth of 1-2-3 superconductor films and possesses desirable rf properties of low dielectric constant and loss tangent. The lattice constant of 3.792 A(ring) provides a lattice match to within 1% of the a axis of 1-2-3. Sputtered films of erbium-barium-copper-oxide have been produced on (100) LaAlO3 substrates that exhibit sharp resistive transitions at 90 K (ΔT=1K), bulk superconductivity as determined by ac susceptibility measurements, and nearly single-crystal growth as evidenced by x-ray diffraction and high-resolution scanning electron microscopy. The high-frequency dielectric properties of LaAlO3 were experimentally investigated at several temperatures. The low-frequency dielectric constant was measured to be 15 and the microwave loss tangent ranged from 6×10−4 at room temperature to 5×10−6 at 4 K.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.100543
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  • 8
    Electronic Resource
    Electronic Resource
    Characteristics of high performance YBa2Cu3O7 step-edge junctions (1992)
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 61 (1992), S. 1128-1130 
    Details
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Step-edge Josephson junctions are engineered grain boundary junctions fabricated using standard lithographic and film deposition techniques. We report a systematic study of 180 YBa2Cu3O7 step-edge junctions and identify a fabrication technique which results in a 90% yield of working junctions with critical current spreads from 30% to 50% (1σ/Ic-ave)over the entire substrate. Technically useful critical current values at 65 K can be obtained by adjusting YBa2Cu3O7 film thickness. IcRn values, approximately independent of film thickness, are ∼1 mV at 4.2 K and ∼0.1 mV at 65 K. Most junctions exhibit ideal electrical behavior in accordance with the RSJ model.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.107690
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  • 9
    Electronic Resource
    Electronic Resource
    Microstrip resonators using two-sided metalorganic chemical vapor deposited Er-Ba-Cu-O thin films (1991)
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 58 (1991), S. 1109-1111 
    Details
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: We have measured very low microwave losses in microstrip resonators produced in a newly developed process which deposits high-temperature Er-Ba-Cu-O superconducting films by metalorganic chemical vapor deposition on both sides of a LaAlO3 substrate. These in situ films have critical temperatures of 92 K. High Tc superconductor (HTS) linear microstrip resonators demonstrated Q's of 3500 at 10 GHz and 4.2 K, corresponding to surface resistances less than 330 μΩ, which is 45 times better than measured silver resoantors. HTS meanderline resonators with a fundamental frequency of 1.3 GHz have Q's of 9600 at 4.2 K. Identical silver meanderline circuits have 300 times more surface resistance than HTS circuits.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.104387
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  • 10
    Electronic Resource
    Electronic Resource
    Substrate step-edge YBa2Cu3O7 rf SQUIDs (1991)
    Woodbury, NY : American Institute of Physics (AIP)
    Applied Physics Letters 58 (1991), S. 543-545 
    Details
    ISSN: 1077-3118
    Source: AIP Digital Archive
    Topics: Physics
    Notes: We have developed a technique which permits high-yield fabrication of microbridges and low noise YBa2Cu3O7 superconducting quantum interference devices (SQUIDs) in epitaxial thin films. These SQUIDs operate over a wide temperature range extending from 4 K to close to the superconducting transition temperature. Measurements of an rf SQUID operating at 77 K give a peak-to-peak flux sensitivity of 36 μV/Φ0 and a flux noise at 10 Hz of 1.5× 10−4 Φ0/(square root of)Hz. Device yields over 80% have been obtained.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1063/1.104581
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