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  • 1
    Publication Date: 2012-02-15
    Description: We recently implicated two recurrent somatic mutations in an adrenal potassium channel, KCNJ5, as a cause of aldosterone-producing adrenal adenomas (APAs) and one inherited KCNJ5 mutation in a Mendelian form of early severe hypertension with massive adrenal hyperplasia. The mutations identified all altered the channel selectivity filter, producing increased Na+ conductance and membrane depolarization, the signal for aldosterone production and proliferation of adrenal glomerulosa cells. We report herein members of four kindreds with early onset primary aldosteronism of unknown cause. Sequencing of KCNJ5 revealed that affected members of two kindreds had KCNJ5G151R mutations, identical to one of the prevalent recurrent mutations in APAs. These individuals had severe progressive aldosteronism and hyperplasia requiring bilateral adrenalectomy in childhood for blood pressure control. Affected members of the other two kindreds had KCNJ5G151E mutations, which are not seen in APAs. These subjects had easily controlled hypertension and no evidence of hyperplasia. Surprisingly, electrophysiology of channels expressed in 293T cells demonstrated that KCNJ5G151E was the more extreme mutation, producing a much larger Na+ conductance than KCNJ5G151R, resulting in rapid Na+-dependent cell lethality. We infer that this increased lethality limits adrenocortical cell mass and the severity of aldosteronism in vivo, accounting for the milder phenotype among these patients. These findings demonstrate striking variations in phenotypes and clinical outcome resulting from different mutations of the same amino acid in KCNJ5 and have implications for the diagnosis and pathogenesis of primary aldosteronism with and without adrenal hyperplasia.
    Print ISSN: 0027-8424
    Electronic ISSN: 1091-6490
    Topics: Biology , Medicine , Natural Sciences in General
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Optical and quantum electronics 7 (1975), S. 115-119 
    ISSN: 1572-817X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Electrical Engineering, Measurement and Control Technology , Physics
    Notes: Abstract Design considerations and performance of a prism beam expander are presented. Using a prism beam expander and holographic grating, a dye laser pumped by a nitrogen laser has given 15 kW of diffraction limited power in 0.1–0.2 cm−1 linewidth. Addition of a single etalon gave a single frequency output of 10 kW in a linewidth of less than 0.01 cm−1.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Applied physics 27 (1982), S. 175-176 
    ISSN: 1432-0649
    Keywords: 42.65C
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract 6s–9s electronic Raman scattering in caesium vapour is used to generate tunable 16μm radiation. Output energies in excess of 8 μJ were obtained, with peak powers in the kilowatt range.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Applied physics 8 (1975), S. 333-340 
    ISSN: 1432-0630
    Keywords: Tunable lasers ; Infrared ; Stimulated Raman effect ; Gain focussing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract It is shown that for generation of infrared radiation by stimulated Raman scattering, the diffraction spread of the Stokes wave can have a significant effect on the threshold. Compared with an analysis in which gain focussing is neglected, the threshold powers may be much higher with a corresponding reduction in tuning range. The design of a Raman oscillator is considerably influenced by these diffraction effects, and also it is found that the Stokes wave is subject to frequency-pulling which is dependent on the pump power.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Applied physics 7 (1975), S. 215-226 
    ISSN: 1432-0630
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract Pulsed laser damage thresholds have been measured for proustite (Ag3 As S3) as the wavelengths 0.694, 1.065, 1.32 and 10.6 μm. The damage thresholds have been found to vary with both the wavelength and duration of the irradiating pulse. At 1.065 μm damage thresholds are 0.38 J/cm2 for pulses of duration 〈50 ns whilst for durations 〉50 ns a value of 7 MW/cm2 is appropriate. The results suggest that damage is initiated by absorbing inclusions approximately 0.6 μm in diameter embedded within the crystals. These inclusions are heated by an incident pulse to cause catastrophic damage of both the surface and interior of an irradiated sample. A model has been developed to enable a study of the thermal behaviour of inclusions irradiated by laser pulses with Gaussian time-dependence to be made.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Small business economics 2 (1990), S. 155-162 
    ISSN: 1573-0913
    Source: Springer Online Journal Archives 1860-2000
    Topics: Economics
    Notes: Abstract This study examines the relationship of bank participation in SBA guaranteed lending programs to bank characteristics and market demographic characteristics. Band SBA lending is related to per capita income in the bank's market area, membership in a bank holding company, the loan-deposit ratio of the bank, the percent business loans of the bank, and the total assets of the bank. In markets of 50,000 or less, greater SBA lending is associated with lower per capita income levels. Accordingly, it appears that the economic development function of bank SBA lending is linked to a greater extent with market areas of less than 50,000.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Small business economics 5 (1993), S. 215-220 
    ISSN: 1573-0913
    Source: Springer Online Journal Archives 1860-2000
    Topics: Economics
    Notes: Abstract Securitization involves the repackaging of individual SBA Guaranteed loans made by financial institutions into pools of varying maturities for resale to intermediaries and final investors. Pool volume has been increasing at a rapid rate since the program's inception in 1985. National and regional investment companies, acting as intermediaries, are the major pool buyers. Investors in pools include banks, savings & loans associations, and pension funds. Securitization has resulted in a major secondary market for institutions that make SBA Guaranteed loans. Public policy goals are served by the increase in SBA lending activity that appears related to the securitization process.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Applied physics 21 (1980), S. 199-204 
    ISSN: 1432-0630
    Keywords: 42.60 ; 42.65
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract Coherent infrared radiation tunable between 1.6 and 3.2 μm was generated with a pulsed dye laser by 6s−5d 5/2 stimulated electronic Raman scattering in caesium vapour. We describe a technique using thermal dissociation to reduce the density of caesium dimers in a heat-pipe oven, which produced a factor of 7 reduction in Raman threshold. The threshold displayed a marked dependence on pump bandwidth.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Applied physics 21 (1980), S. 353-359 
    ISSN: 1432-0630
    Keywords: 42.10 ; 42.60 ; 42.80
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract Some aspects of the transient behaviour of pulsed dye lasers are investigated both theoretically and experimentally. Relaxation oscillations induced in the output from a dye cell by an external reflector have been observed and explained, using a rate equation approximation to the physical situation in the dye cell. These oscillations are shown to play an important part in the generation of short laser pulses from long cavity dye lasers. Finally, it is proposed that 10 ps pulses can be obtained from conventional dye lasers pumped by 100 ps pulses from a high-pressure nitrogen laser.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0630
    Keywords: 06.60 Jn
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract An extension of the noncollinear second-harmonic generation technique for pulse autocorrelation measurements is described. A diffraction grating is used to produce a tailored, expanded beam, with a differential time delay along its expanded axis. When this beam is combined with an inverted replica of itself at the frequency-doubling crystal, the monitored spatial profile of the generated second-harmonic beam gives directly the duration of the incident laser pulse. A time resolution of better than 1 ps is obtained at 500 nm, and a total measurement range of∼80 ps. The optical system here described enables the extension of the measurement range in a simple manner.
    Type of Medium: Electronic Resource
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