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991.
Carbohydrate-deficient-glycoprotein syndrome type 1 (CDG1; also known as "Jaeken syndrome") is an autosomal recessive disorder characterized by defective glycosylation. Most patients show a deficiency of phosphomannomutase (PMM), the enzyme that converts mannose 6-phosphate to mannose 1-phosphate in the synthesis of GDP-mannose. The disease is linked to chromosome 16p13, and mutations have recently been identified in the PMM2 gene in CDG1 patients with a PMM deficiency (CDG1A). The availability of the genomic sequences of PMM2 allowed us to screen for mutations in 56 CDG1 patients from different geographic origins. By SSCP analysis and by sequencing, we identified 23 different missense mutations and 1 single-base-pair deletion. In total, mutations were found on 99% of the disease chromosomes in CDG1A patients. The R141H substitution is present on 43 of the 112 disease alleles. However, this mutation was never observed in the homozygous state, suggesting that homozygosity for these alterations is incompatible with life. On the other hand, patients were found homozygous for the D65Y and F119L mutations, which must therefore be mild mutations. One particular genotype, R141H/D188G, which is prevalent in Belgium and the Netherlands, is associated with a severe phenotype and a high mortality. Apart from this, there is only a limited relation between the genotype and the clinical phenotype. 相似文献
992.
We investigated accuracy in discriminating between constant and variable angular velocities for orthographic projections of three-dimensional rotating objects. The reported judgments of "constant" or "variable" angular velocity were only slightly influenced by the projected angular velocities, but they were greatly affected by the variations of the deformation, a first-order component of the optic flow. When viewing either a rotating ellipsoidal volume or a planar surface that accelerated and decelerated over the course of rotation, observers' tendencies to report a variable angular velocity were increased when the temporal phase of the acceleration pattern increased the range of variation of the median deformation; the tendencies were decreased when the same acceleration pattern was used to decrease the range of variation of the median deformation. These results provide evidence contrary to the hypothesis that the visual system performs a mathematically correct analysis of the optic flow. 相似文献
993.
Forked laser eigenstates are shown to provide a powerful tool both to phase lock spatially separated laser oscillators and to add their powers coherently into a TEM(00) output beam. Coherent addition of the powers extracted from two fiber-coupled diode-pumped Nd:YAG channels is theoretically and experimentally demonstrated. Pure TEM(00) oscillation is obtained with a 20% optical-to-optical conversion efficiency. The coherence of the two-propagation-axis laser is proved, and single-frequency operation is demonstrated. The scalability of the scheme is discussed. 相似文献
994.
Fluid jet polishing of optical surfaces 总被引:8,自引:0,他引:8
We present a new finishing process that is capable of locally shaping and polishing optical surfaces of complex shapes. A fluid jet system is used to guide a premixed slurry at pressures less than 6 bars to the optical surface. We used a slurry comprising water and 10% #800 SiC abrasives (21.8 mum) to reduce the surface roughness of a BK7 sample from 350 to 25 nm rms and to vary the shape of a polished sample BK7, maintaining its surface roughness of 1.6 nm rms, thereby proving both the shaping and polishing possibilities of the presented method. 相似文献
995.
Measurement of transient deformations with dual-pulse addition electronic speckle-pattern interferometry 总被引:3,自引:0,他引:3
We describe an electronic speckle-pattern interferometry system for analyzing addition fringes generated by the transient deformation of a test object. The system is based on a frequency-doubled twin Nd:YAG laser emitting dual pulses at a TV camera field rate (50 Hz). The main advance has been the automatic, quantitative analysis of dual-pulse addition electronic speckle-pattern interferometry data by the introduction of carrier fringes and the application of Fourier methods. The carrier fringes are introduced between dual pulses by a rotating mirror that tilts the reference beam. The resulting deformation-modulated addition fringes are enhanced with a deviation filter, giving fringe visibility close to that of subtraction fringes. The phase distribution is evaluated with a Fourier-transform method with bandpass filtering. From the wrapped phase distribution, a continuous phase map is reconstructed with an iterative weighted least-squares unwrapper. Preliminary results for a thin plate excited by an acoustic shock show the suitability of the system for the quantitative evaluation of transient deformation fields. 相似文献
996.
We present a multiple-input, single-output, weakly nonlinear model of spatial light modulators by use of a second-order Volterra series and describe an experimental method to measure the nonlinear transfer functions by means of sinusoidal perturbation and synchronous detection with a lock-in amplifier. We also present an application of this method to a liquid-crystal light valve. 相似文献
997.
Acousto-optic (AO) devices are important spatial light modulators. They can be used as light-beam deflectors, rf true-time-delay lines, etc. To increase their spur-free dynamic range, we present what to our knowledge is a novel multichannel AO device structure, in which different channels have different carrier frequencies, so a wideband signal can automatically be decomposed into a set of narrow-band signals. Design, fabrication, and testing of this 24-channel, 10-mus AO spatial light modulator are addressed. 相似文献
998.
A laser Doppler velocimeter employing a compact disc pickup for both fringe projection and signal detection is described. The spectrum of the recorded signal gives the information about the speed of the object. The device takes advantage of the Talbot effect to project the grating contained in the pickup onto a moving target, so that no imaging system is required. The peculiar imaging technique allows for the exploitation of several optical configurations and permits the manipulation of the intensity profile of the projected grating. The instrument was used to measure the velocity of dust particles on a solid substrate in the 1-m/s range but could also find an application to the study of liquid flow. 相似文献
999.
Picosecond pulses emitted from a gain-switched laser diode have been amplified in a Ti:sapphire regenerative amplifier indirectly pumped by a 4-W laser diode. This all-solid-state system produced microjoule pulses tunable from 803 to 840 nm at repetition rates up to 25 kHz with durations of 70-100 ps. By frequency doubling and tripling the output, we generated blue and UV pulses tunable from 401 to 420 nm and from 268 to 280 nm, respectively. Average powers larger than 4 mW were reached in these two wavelength regions. 相似文献
1000.
Scattering effects in a two-photon optical data storage system are numerically studied. Surface scattering analysis with a scalar, beam propagation model is performed. We analyze the problem by modeling scattering from randomly varying surfaces and also by Fourier surface decomposition. Scattering induced by propagation through multiple pages of randomly recorded data marks is also studied with a hybrid finite-difference-time-domain/angular-spectrum model. Both surface and bulk scattering are shown to influence the spatial properties of the optical beam. Results and some possible implications are presented. 相似文献