556 research outputs found
Space telescope phase B definition study. Volume 2A: Science instruments, astrometer
The analysis and design of an astrometer instrument for the space telescope are discussed. The design concepts utilize the astrometric multiplexing area scanner and the OTA fine guidance sensor
Real-time sensing of optical alignment
The Large Deployable Reflector and other future segmented optical systems may require autonomous, real-time alignment of their optical surfaces. Researchers have developed gratings located directly on a mirror surface to provide interferometric sensing of the location and figure of the mirror. The grating diffracts a small portion of the incident beam to a diffractive focus where the designed diagnostics can be performed. Mirrors with diffraction gratings were fabricated in two separate ways. The formation of a holographic grating over the entire surface of a mirror, thereby forming a Zone Plate Mirror (ZPM) is described. Researchers have also used computer-generated hologram (CGH) patches for alignment and figure sensing of mirrors. When appropriately illuminated, a grid of patches spread over a mirror segment will yield a grid of point images at a wavefront sensor, with the relative location of the points providing information on the figure and location of the mirror. A particular advantage of using the CGH approach is that the holographic patches can be computed, fabricated, and replicated on a mirror segment in a mass production 1-g clean room environment
Theoretical efficiency of the Princeton two-element echelle spectrograph
Echelle spectrometer for use with spaceborne stellar telescope in Advanced Princeton Satellite Stud
On the regional climatic impact of contrails: microphysical and radiative properties of contrails and natural cirrus clouds
International audienceThe impact of contrail-induced cirrus clouds on regional climate is estimated for mean atmospheric conditions of southern Germany in the months of July and October. This is done by use of a regionalized one-dimensional radiative convective model (RCM). The influence of an increased ice cloud cover is studied by comparing RCM results representing climatological values with a modified case. In order to study the sensitivity of this effect on the radiative characteristics of the ice cloud, two types of additional ice clouds were modelled: cirrus and contrails, the latter cloud type containing a higher number of smaller and less of the larger cloud particles. Ice cloud parameters are calculated on the basis of a particle size distribution which covers the range from 2 to 2000 µm, taking into consideration recent measurements which show a remarkable amount of particles smaller than 20 µm. It turns out that a 10% increase in ice cloud cover leads to a surface temperature increase in the order of 1K, ranging from 1.1 to 1.2K in July and from 0.8 to 0.9K in October depending on the radiative characteristics of the air-traffic-induced ice clouds. Modelling the current contrail cloud cover which is near 0.5% over Europe yields a surface temperature increase in the order of 0.05K
Anisotropic splitting of intersubband spin plasmons in quantum wells with bulk and structural inversion asymmetry
In semiconductor heterostructures, bulk and structural inversion asymmetry
and spin-orbit coupling induce a k-dependent spin splitting of valence and
conduction subbands, which can be viewed as being caused by momentum-dependent
crystal magnetic fields. This paper studies the influence of these effective
magnetic fields on the intersubband spin dynamics in an asymmetric n-type
GaAs/AlGaAs quantum well. We calculate the dispersions of intersubband spin
plasmons using linear response theory. The so-called D'yakonov-Perel'
decoherence mechanism is inactive for collective intersubband excitations,
i.e., crystal magnetic fields do not lead to decoherence of spin plasmons.
Instead, we predict that the main signature of bulk and structural inversion
asymmetry in intersubband spin dynamics is a three-fold, anisotropic splitting
of the spin plasmon dispersion. The importance of many-body effects is pointed
out, and conditions for experimental observation with inelastic light
scattering are discussed.Comment: 8 pages, 6 figure
Spin Orientation and Spin Precession in Inversion-Asymmetric Quasi Two-Dimensional Electron Systems
Inversion asymmetry induced spin splitting of the electron states in quasi
two-dimensional (2D) systems can be attributed to an effective magnetic field B
which varies in magnitude and orientation as a function of the in-plane wave
vector k||. Using a realistic 8x8 Kane model that fully takes into account spin
splitting because of both bulk inversion asymmetry and structure inversion
asymmetry we investigate the spin orientation and the effective field B for
different configurations of a quasi 2D electron system. It is shown that these
quantities depend sensitively on the crystallographic direction in which the
quasi 2D system was grown as well as on the magnitude and orientation of the
in-plane wave vector k||. These results are used to discuss how spin-polarized
electrons can precess in the field B(k||). As a specific example we consider
GaInAs-InP quantum wells.Comment: 10 pages, 6 figure
Novel C8orf37 mutations cause retinitis pigmentosa in consanguineous families of Pakistani origin
Purpose: To investigate the molecular basis of retinitis pigmentosa in two consanguineous families of Pakistani origin with multiple affected members. Methods: Homozygosity mapping and Sanger sequencing of candidate genes were performed in one family while the other was analyzed with whole exome next-generation sequencing. A minigene splicing assay was used to confirm the splicing defects. Results: In family MA48, a novel homozygous nucleotide substitution in C8orf37, c.244–2A>C, that disrupted the consensus splice acceptor site of exon 3 was found. The minigene splicing assay revealed that this mutation activated a cryptic splice site within exon 3, causing a 22 bp deletion in the transcript that is predicted to lead to a frameshift followed by premature protein truncation. In family MA13, a novel homozygous null mutation in C8orf37, c.555G>A, p.W185*, was identified. Both mutations segregated with the disease phenotype as expected in a recessive manner and were absent in 8,244 unrelated individuals of South Asian origin. Conclusions: In this report, we describe C8orf37 mutations that cause retinal dystrophy in two families of Pakistani origin, contributing further data on the phenotype and the spectrum of mutations in this form of retinitis pigmentosa
Relative frequencies of inherited retinal dystrophies and optic neuropathies in Southern France: assessment of 21-year data management
PURPOSE: Inherited retinal dystrophies (IRDs) and inherited optic neuropathies (IONs) are rare diseases defined by specific clinical and molecular features. The relative prevalence of these conditions was determined in Southern France.
METHODS: Patients recruited from a specialized outpatient clinic over a 21-year period underwent extensive clinical investigations and 107 genes were screened by polymerase chain reaction/sequencing.
RESULTS: There were 1957 IRD cases (1481 families) distributed in 70% of pigmentary retinopathy cases (56% non-syndromic, 14% syndromic), 20% maculopathies and 7% stationary conditions. Patients with retinitis pigmentosa were the most frequent (47%) followed by Usher syndrome (10.8%). Among non-syndromic pigmentary retinopathy patients, 84% had rod-cone dystrophy, 8% cone-rod dystrophy and 5% Leber congenital amaurosis. Macular dystrophies were encountered in 398 cases (30% had Stargardt disease and 11% had Best disease). There were 184 ION cases (127 families) distributed in 51% with dominant optic neuropathies, 33% with recessive/sporadic forms and 16% with Leber hereditary optic neuropathy. Positive molecular results were obtained in 417/609 families with IRDs (68.5%) and in 27/58 with IONs (46.5%). The sequencing of 5 genes (ABCA4, USH2A, MYO7A, RPGR and PRPH2) provided a positive molecular result in 48% of 417 families with IRDs. Except for autosomal retinitis pigmentosa, in which less than half the families had positive molecular results, about 75% of families with other forms of retinal conditions had a positive molecular diagnosis.
CONCLUSIONS: Although gene discovery considerably improved molecular diagnosis in many subgroups of IRDs and IONs, retinitis pigmentosa, accounting for almost half of IRDs, remains only partly molecularly defined
De novo intrachromosomal gene conversion from OPN1MW to OPN1LW in the male germline results in Blue Cone Monochromacy
X-linked cone dysfunction disorders such as Blue Cone Monochromacy and
X-linked Cone Dystrophy are characterized by complete loss (of) or reduced L-
and M- cone function due to defects in the OPN1LW/OPN1MW gene cluster. Here we
investigated 24 affected males from 16 families with either a structurally
intact gene cluster or at least one intact single (hybrid) gene but harbouring
rare combinations of common SNPs in exon 3 in single or multiple OPN1LW and
OPN1MW gene copies. We assessed twelve different OPN1LW/MW exon 3 haplotypes
by semi-quantitative minigene splicing assay. Nine haplotypes resulted in
aberrant splicing of ≥20% of transcripts including the known pathogenic
haplotypes (i.e. ‘LIAVA’, ‘LVAVA’) with absent or minute amounts of correctly
spliced transcripts, respectively. De novo formation of the ‘LIAVA’ haplotype
derived from an ancestral less deleterious ‘LIAVS’ haplotype was observed in
one family with strikingly different phenotypes among affected family members.
We could establish intrachromosomal gene conversion in the male germline as
underlying mechanism. Gene conversion in the OPN1LW/OPN1MW genes has been
postulated, however, we are first to demonstrate a de novo gene conversion
within the lineage of a pedigree
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