To characterize developmental defects and the time course of Norrie disease in retinal and hyaloid vasculature during retinal development and to identify underlying molecular angiogenic pathways that ...may be affected in Norrie disease, exudative vitreoretinopathy, retinopathy of prematurity, and Coats' disease.
Norrie disease pseudoglioma homologue (Ndph)-knockout mice were studied during retinal development at early postnatal (p) stages (p5, p10, p15, and p21). Histologic techniques, quantitative RT-PCR, ELISA, and Western blot analyses provided molecular data, and scanning laser ophthalmoscopy (SLO) angiography and electroretinography (ERG) were used to obtain in vivo data.
The data showed that regression of the hyaloid vasculature of Ndph-knockout mice occurred but was drastically delayed. The development of the superficial retinal vasculature was strongly delayed, whereas the deep retinal vasculature did not form because of the blockage of vessel outgrowth into the deep retinal layers. Subsequently, microaneurysm-like lesions formed. Several angiogenic factors were differentially transcribed during retinal development. Increased levels of hypoxia inducible factor-1alpha (HIF1alpha) and VEGFA, as well as a characteristic ERG pattern, confirmed hypoxic conditions in the inner retina of the Ndph-knockout mouse.
These data provide evidence for a crucial role of Norrin in hyaloid vessel regression and in sprouting angiogenesis during retinal vascular development, especially in the development of the deep retinal capillary networks. They also suggest an early and a late phase of Norrie disease and may provide an explanation for similar phenotypic features of allelic retinal diseases in mice and patients as secondary consequences of pathologic hypoxia.
Overview of the CDF Run II data handling system Buckley-Geer, L.; Lammel, S.; Leininger, M. ...
Computer physics communications,
10/2001, Letnik:
140, Številka:
1
Journal Article, Conference Proceeding
Recenzirano
The Collider Detector at Fermilab (CDF) collaboration records and analyses proton–anti-proton interactions with a center-of-mass energy of 2 TeV at the Tevatron. During the next collider run the ...experiment expects to record about 1 PetaByte of data, an increase by more than an order of magnitude in data volume compared to the existing data of the experiment.
This paper gives an overview of the new data handling system. The design builds upon successful strategies used in the past but eliminates shortcomings encountered with the old system. The core of the central analysis system will be a pool of over 20 TBytes of data disks. Logically behind this disk pool will be an automated tape library with a storage capacity to hold the full 1 PByte of Run II data. Multi-processor compute systems will be located around this storage pool to provide the required analysis power of over 3000 SPECint95. Desktop systems will be integrated into the analysis system via a shared login area and user authentication. The data handling software falls into four categories: software to manage computing resources, software for the data disks and for the mass storage subsystem, software to organize and manage the meta-data information, and the I/O modules in the analysis framework for accessing data.
In this study we examine five lunar domes in Mare Undarum. Four domes termed Condorcet 1–4 are located between the craters Condorcet P and Dubiago, immediately east of Dubiago V and W. The fifth ...dome, termed Dubiago 3, is located about 35
km further south. The region under study is situated in a major trough concentric to the Crisium basin. The domes Condorcet 1–3 are aligned radially with respect to the Crisium basin. Similar dome configurations aligned radial to major impact basins are known from other lunar mare dome fields. The spectral signature of the domes derived from Clementine UV/VIS imagery reveals that they consist of basaltic lava with a low TiO
2 content below 2
wt% and with a FeO content around 10
wt%. Three examined domes exhibit highland components in their soils, which we attribute to lateral mixing between the material in the mare ponds and the surrounding highland material due to random impacts. All five domes have moderate diameters between 10 and 12
km. Condorcet 1–3 are similar to effusive domes of intermediate flank slope between
1
∘
and
2
∘
like those situated in the Hortensius/Milichius/T. Mayer region, while Condorcet 4 has an exceptionally steep flank slope of
2
.
8
∘
and a large volume. With its low flank slope of
0
.
9
∘
, the dome Dubiago 3 is morphometrically very similar to a known intrusive dome in the west of Mare Serenitatis. Hence, this structure is possibly of intrusive origin, but with the available data an effusive origin cannot be ruled out. Based on a rheologic model, we infer the physical conditions under which the domes were formed (lava viscosity, effusion rate, magma rise speed) as well as the geometries of the feeder dikes.
Computing models of CDF and DØ in Run II Lammel, Stephan
Computer physics communications,
05/1998, Letnik:
110, Številka:
1-3
Journal Article, Conference Proceeding
Recenzirano
Odprti dostop
The next collider run of the Fermilab Tevatron, Run II, is scheduled for autumn of 1999. Both experiments, the Collider Detector at Fermilab (CDF) and the DØ experiment are being modified to cope ...with the higher luminosity and shorter bunchspacing of the Tevatron. New detector components, higher event complexity, and an increased data volume require changes from the data acquisition systems up to the analysis systems. In this paper we present a summary of the computing models of the two experiments for Run II.
While frame-shift mutations are usually found in Duchenne muscular dystrophy (DMD), in-frame mutations are associated with the less severe phenotype of Becker's muscular dystrophy. Exceptions have ...been reported in both directions suggesting the existence of modifying genes, which might be helpful for innovation of new therapeutic strategies. We report on the very rare case of an intrafamilially different course of DMD, with the younger brother being far less affected than the older one when compared at the same age. In this context, we constructed a subtraction library enriched for transcripts over-expressed in the patient with the milder phenotype. Twelve random clones were sequenced, followed by database analysis. Six of them, casein kinase 1 alpha 1, RAP2B, dynactin 3 light chain, core binding factor beta, myosin light polypeptide 2 and one hypothetical gene, were further analysed by real-time RT-PCR. All these genes were over-expressed 3-20 times in the less affected patient compared with the more severely affected one. Casein kinase 1 and the hypothetical gene showed even a slightly higher expression than the control. Up-regulation of myosin light polypeptide 2, one of the most sensitive markers of muscle fibre regeneration, obviously reflects the milder phenotype. Casein kinase 1, dynactin and core binding factor are supposed to be involved in cell cycle pathways. RAP is a component of the signalling network which controls fundamental cellular processes such as proliferation and differentiation. All four might be interesting candidates for a therapeutic approach to diminish progression of dystrophy in DMD.
We report the results of a search for a W' boson produced in pp collisions at a center-of-mass energy of 1.8 TeV using a 107 pb-1 data sample recorded by the Collider Detector at Fermilab. We ...consider the decay channel W'-->&munumu and search for anomalous production of high transverse mass munumu lepton pairs. We observe no excess of events above background and set limits on the rate of W' boson production and decay relative to standard model W boson production and decay using a fit of the transverse mass distribution observed. If we assume standard model strength couplings of the W' boson to quark and lepton pairs, we exclude a W' boson with invariant mass less than 660 GeV/c2 at 95% confidence level.
We have studied the production of B hadrons in 1.8-TeV ppover ¯ collisions. We present measurements of the fragmentation fractions, f_{u}, f_{d}, f_{s}, and f_{baryon}, of produced b quarks that ...yield B^{+}, B^{0}, B_{s}^{0}, and Λover ¯_{b}^{0} hadrons. Reconstruction of five electron-charm final states yields f_{s}/( f_{u}+f_{d})=0.213±0.068 and f_{baryon}/( f_{u}+f_{d})=0.118±0.042, assuming f_{u}=f_{d}. If all B hadrons produced in ppover ¯ collisions cascade to one of these four hadrons, we determine f_{u}=f_{d}=0.375±0.023, f_{s}=0.160±0.044, and f_{baryon}=0.090±0.029. If we do not assume f_{u}=f_{d}, we find f_{d}/f_{u}=0.84±0.16.
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