Double Concentric Autofluorescence Ring in NR2E3-p.G56R-linked Autosomal Dominant Retinitis Pigmentosa.

Purpose: We report an unusual appearance of fundus autofluorescence (FAF) associated with NR2E3-p.G56R-linked autosomal dominant retinitis pigmentosa (ADRP).Methods: Patients were enrolled among three generations in a Swiss family. Molecular diagnosis identified a c.166G>A (p.G56R) mutation. Ophthalmic examination included fundus photography, FAF, near-infrared autofluorescence (NIA), optical coherence tomography (OCT) and visual fields (VF).Results: Fundus examination revealed a wide range of features from unremarkable to attenuated arterial caliber, clumped and spicular pigment deposits in the mid-periphery and optic nerve pallor. FAF showed a double concentric hyperautofluorescent ring: an inner perimacular ring which tended to be smaller in older patients, and an outer ring located along the vascular arcades, which appeared to extend over time towards the periphery and eventually became hypoautofluorescent. The inner and outer hyperautofluorescent rings were seen both on NIA and FAF at a similar localization. There was also a spatial correspondence between the loss of photoreceptor inner segment and outer segment junction on OCT and the area delimited by both double FAF and NIA rings. VF showed either midperipheral annular scotoma or constricted visual field loss in advanced cases, correlating with dystrophic non-functional retinal regions demarcated by the hyperautofluorescent annuli. A double ring of hyperautofluorescence was observed in all but one patient of two additional families, but not in patients harboring mutations in other ADRP genes, including PRPF3, RHO, RP1, PRPH2, PROM1 and CTRP5.Conclusions: The presence of a double concentric hyperautofluorescent ring of FAF may represent a highly penetrant early phenotypic marker of NR2E3-p.G56R-linked ADRP.

Development, optimization, and validation of novel anti-TEM1/CD248 affinity agent for optical imaging in cancer.

Tumor Endothelial Marker-1 (TEM1/CD248) is a tumor vascular marker with high therapeutic and diagnostic potentials. Immuno-imaging with TEM1-specific antibodies can help to detect cancerous lesions, monitor tumor responses, and select patients that are most likely to benefit from TEM1-targeted therapies. In particular, near infrared(NIR) optical imaging with biomarker-specific antibodies can provide real-time, tomographic information without exposing the subjects to radioactivity. To maximize the theranostic potential of TEM1, we developed a panel of all human, multivalent Fc-fusion proteins based on a previously identified single chain antibody (scFv78) that recognizes both human and mouse TEM1. By characterizing avidity, stability, and pharmacokinectics, we identified one fusion protein, 78Fc, with desirable characteristics for immuno-imaging applications. The biodistribution of radiolabeled 78Fc showed that this antibody had minimal binding to normal organs, which have low expression of TEM1. Next, we developed a 78Fc-based tracer and tested its performance in different TEM1-expressing mouse models. The NIR imaging and tomography results suggest that the 78Fc-NIR tracer performs well in distinguishing mouse- or human-TEM1 expressing tumor grafts from normal organs and control grafts in vivo. From these results we conclude that further development and optimization of 78Fc as a TEM1-targeted imaging agent for use in clinical settings is warranted.

Survey on batch-to-batch variation in spray paints: a collaborative study

This study represents the most extensive analysis of batch-to-batch variations in spray paint samples to date. The survey was performed as a collaborative project of the ENFSI (European Network of Forensic Science Institutes) Paint and Glass Working Group (EPG) and involved 11 laboratories. Several studies have already shown that paint samples of similar color but from different manufacturers can usually be differentiated using an appropriate analytical sequence. The discrimination of paints from the same manufacturer and color (batch-to-batch variations) is of great interest and these data are seldom found in the literature. This survey concerns the analysis of batches from different color groups (white, papaya (special shade of orange), red and black) with a wide range of analytical techniques and leads to the following conclusions. Colored batch samples are more likely to be differentiated since their pigment composition is more complex (pigment mixtures, added pigments) and therefore subject to variations. These variations may occur during the paint production but may also occur when checking the paint shade in quality control processes. For these samples, techniques aimed at color/pigment(s) characterization (optical microscopy, microspectrophotometry (MSP), Raman spectroscopy) provide better discrimination than techniques aimed at the organic (binder) or inorganic composition (fourier transform infrared spectroscopy (FTIR) or elemental analysis (SEM - scanning electron microscopy and XRF - X-ray fluorescence)). White samples contain mainly titanium dioxide as a pigment and the main differentiation is based on the binder composition (Csingle bondH stretches) detected either by FTIR or Raman. The inorganic composition (elemental analysis) also provides some discrimination. Black samples contain mainly carbon black as a pigment and are problematic with most of the spectroscopic techniques. In this case, pyrolysis-GC/MS represents the best technique to detect differences. Globally, Py-GC/MS may show a high potential of discrimination on all samples but the results are highly dependent on the specific instrumental conditions used. Finally, the discrimination of samples when data was interpreted visually as compared to statistically using principal component analysis (PCA) yielded very similar results. PCA increases sensitivity and could perform better on specific samples, but one first has to ensure that all non-informative variation (baseline deviation) is eliminated by applying correct pre-treatments. Statistical treatments can be used on a large data set and, when combined with an expert's opinion, will provide more objective criteria for decision making.

Faint arc-minute extended radio emission around Cygnus X-3

Aims.We revisit the vicinity of the microquasar Cygnus X-3 at radio wavelengths. We aim to improve our previous search for possible associated extended radio features/hot spots in the position angle of the Cygnus X-3 relativistic jets focusing on shorter angular scales than previously explored. Methods.Our work is mostly based on analyzing modern survey and archive radio data, mainly including observations carried out with the Very Large Array and the Ryle Telescopes. We also used deep near-infrared images that we obtained in 2005. Results.We present new radio maps of the Cygnus X-3 field computed after combining multi-configuration Very Large Array archive data at 6 cm and different observing runs at 2 cm with the Ryle Telescope. These are probably among the deepest radio images of Cygnus X-3 reported to date at cm wavelengths. Both interferometers reveal an extended radio feature within a few arc-minutes of the microquasar position, thus making our detection more credible. Moreover, this extended emission is possibly non-thermal, although this point still needs confirmation. Its physical connection with the microquasar is tentatively considered under different physical scenarios. We also report on the serendipitous discovery of a likely Fanaroff-Riley type II radio galaxy only away from Cygnus X-3.

Effects of prior hydrogenation on the structure and properties of thermally nanocrystallized silicon layers

Nanocrystalline silicon layers have been obtained by thermal annealing of films sputtered in various hydrogen partial pressures. The as-deposited and crystallized films were investigated by infrared, Raman, x-ray diffraction, electron microscopy, and optical absorption techniques. The obtained data show evidence of a close correlation between the microstructure and properties of the processed material, and the hydrogen content in the as-grown deposit. The minimum stress deduced from Raman was found to correspond to the widest band gap and to a maximum hydrogen content in the basic unannealed sample. Such a structure relaxation seems to originate from the so-called "chemical annealing" thought to be due to Si-H2 species, as identified by infrared spectroscopy. The variation of the band gap has been interpreted in terms of the changes in the band tails associated with the disorder which would be induced by stress. Finally, the layers originally deposited with the highest hydrogen pressure show a lowest stress-which does not correlate with the hydrogen content and the optical band gap¿and some texturing. These features are likely related to the presence in these layers of a significant crystalline fraction already before annealing.

Unusual concentration of Early Albian arthropod-bearing amber in the Basque-Cantabrian Basin (El Soplao, Cantabria, Spain): Palaeoenvironmental and paleobiological implications.

The El Soplao site is a recently-discovered Early Albian locality of the Basque-Cantabrian Basin (northern Spain) that has yielded a number of amber pieces with abundant bioinclusions. The amber-bearing deposit occurs in a non-marine to transitional marine siliciclastic unit (Las Peñosas Formation) that is interleaved within a regressive-transgressive, carbonate-dominated Lower Aptian-Upper Albian marine sequence. The Las Peñosas Formation corresponds to the regressive stage of this sequence and in its turn it splits into two smaller regressive-transgressive cycles. The coal and amber-bearing deposits occur in deltaic-estuarine environments developed during the maximum regressive episodes of these smaller regressive-transgressive cycles. The El Soplao amber shows Fourier Transform Infrared Spectroscopy spectra similar to other Spanish Cretaceous ambers and it is characterized by the profusion of sub-aerial, stalactite-like flows. Well-preserved plant cuticles assigned to the conifer genera Frenelopsis and Mirovia are abundant in the beds associated with amber. Leaves of the ginkgoalean genera Nehvizdya and Pseudotorellia also occur occasionally. Bioinclusions mainly consist of fossil insects of the orders Blattaria, Hemiptera, Thysanoptera, Raphidioptera, Neuroptera, Coleoptera, Hymenoptera and Diptera, although some spiders and spider webs have been observed as well. Some insects belong to groups scarce in the fossil record, such as a new morphotype of the wasp Archaeromma (of the family Mymarommatidae) and the biting midge Lebanoculicoides (of the monogeneric subfamily Lebanoculicoidinae). This new amber locality constitutes a very significant finding that will contribute to improving the knowledge and comprehension of the Albian non-marine paleoarthropod fauna.

Chemical and structural characterization of soil humic substances under agroforestry and conventional systems

Studies have proven that the agroforestry systems in the semi-arid region of the State of Ceará, Brazil, induce an increase in soil organic C levels. Notwithstanding, there is no information if this increase also results in qualitative changes in different pools of soil organic matter. The objective of this study was to verify the possible chemical and structural alterations in fulvic and humic acids of a Luvisol in areas adopting agroforestry, traditional intensive cultivation and native forest in a long-term experiment conducted in the semi-arid region of Ceará State, Brazil. The study was conducted in an experimental area of the National Goat Research Center (Embrapa) in Sobral, CE. The following treatments were evaluated: agrosilvopasture (AGP), silvopasture (SILV), intensive cultivation under fallow (ICF), and areas with native forest (NF). Soil fulvic and humic acids fractions were extracted from the 0-6 and 6-12 cm layers and characterized by elemental composition, thermogravimetry and infrared spectroscopy analyses. The elemental composition analysis of humic acids confirmed the data found for fulvic acids, showing reduction in the C, H and N levels, followed by an increase in O contents in the AGP and ICF treatments over SILV and NF. In all treatments, except to SILV in the 0-6 cm layer, the percentage of mass loss was highest (300-600 °C) for humic acids in the thermally most stable region. Despite the similarity between infrared spectra, soil fulvic acids in the SILV treatment extracted from 6-12 cm depth decrease the absorption bands at 1708 and 1408 cm-1 followed by an increase in the absorption band at 1608 cm-1 attributed to aromatic C=C groups. This behavior suggests an increase in the aromatic character of the structure. The AGP and ICF treatments, which increase the soil tilling, favored the maintenance of humic substances with a more aromatic character in the soil than SILV and NF. The less aromatic humic substances in the SILV treatment resulted in an increase of exchange sites of soil organic matter, indicating improved nutrient cycling and maintenance of productivity in the system.

Microbiological and biochemical properties of an agricultural Mexican soil amended with sewage sludge

The application of sewage sludge is a concern because it may affect the quality of organic matter and microbiological and biochemical soil properties. The effects of surface application of sewage sludge to an agricultural soil (at 18 and 36 t ha-1 dry basis) were assessed in one maize (Zea mays L.) growing season. The study evaluated microbial biomass, basal respiration and selected enzymatic activities (catalase, urease, acid and alkaline phosphatase, and β-glucosidase) 230 days after sewage sludge application and infrared spectroscopy was used to assess the quality of dissolved organic matter and humic acids. Sewage sludge applications increased the band intensity assigned to polysaccharides, carboxylic acids, amides and lignin groups in the soil. The organic matter from the sewage sludge had a significant influence on the soil microbial biomass; nevertheless, at the end of the experiment the equilibrium of the soil microbial biomass (defined as microbial metabolic quotient, qCO2) was recovered. Soil urease, acid and alkaline phosphatase activity were strongly influenced by sewage sludge applications.

Calorimetry of hydrogen desorption from a-Si nanoparticles

The process of hydrogen desorption from amorphous silicon (a-Si) nanoparticles grown by plasma-enhanced chemical vapor deposition (PECVD) has been analyzed by differential scanning calorimetry (DSC), mass spectrometry, and infrared spectroscopy, with the aim of quantifying the energy exchanged. Two exothermic peaks centered at 330 and 410 C have been detected with energies per H atom of about 50 meV. This value has been compared with the results of theoretical calculations and is found to agree with the dissociation energy of Si-H groups of about 3.25 eV per H atom, provided that the formation energy per dangling bond in a-Si is about 1.15 eV. It is shown that this result is valid for a-Si:H films, too.

Influence of pressure and radio frequency power on deposition rate and structural properties of hydrogenated amorphous silicon thin films prepared by plasma deposition

The influence of radio frequency (rf) power and pressure on deposition rate and structural properties of hydrogenated amorphous silicon (a-Si:H) thin films, prepared by rf glow discharge decomposition of silane, have been studied by phase modulated ellipsometry and Fourier transform infrared spectroscopy. It has been found two pressure regions separated by a threshold value around 20 Pa where the deposition rate increases suddenly. This behavior is more marked as rf power rises and reflects the transition between two rf discharges regimes. The best quality films have been obtained at low pressure and at low rf power but with deposition rates below 0.2 nm/s. In the high pressure region, the enhancement of deposition rate as rf power increases first gives rise to a reduction of film density and an increase of content of hydrogen bonded in polyhydride form because of plasma polymerization reactions. Further rise of rf power leads to a decrease of polyhydride bonding and the material density remains unchanged, thus allowing the growth of a-Si:H films at deposition rates above 1 nm/s without any important detriment of material quality. This overcoming of deposition rate limitation has been ascribed to the beneficial effects of ion bombardment on the a-Si:H growing surface by enhancing the surface mobility of adsorbed reactive species and by eliminating hydrogen bonded in polyhydride configurations.

Organic Matter Fractions and Quality of the Surface Layer of a Constructed and Vegetated Soil After Coal Mining. I - Humic Substances and Chemical Characterization

After open coal mining, soils are “constructed”, which usually contain low levels and quality of organic matter (OM). Therefore, the use of plant species for revegetation and reclamation of degraded areas is essential. This study evaluated the distribution of carbon (C) in the chemical fractions as well as the chemical characteristics and humification degree of OM in a soil constructed after coal mining under cultivation of perennial grasses. The experiment was established in 2003 with the following treatments: Hemarthria altissima (T1), Paspalum notatum (T2), Cynodon dactilon (T3), Urochloa brizantha (T4), bare constructed soil (T5), and natural soil (T6). In 2009, soil samples were collected from the 0.00-0.03 m layer and the total organic carbon stock (TOC) and C stock in the chemical fractions: acid extract (CHCl), fulvic acid (CFA), humic acid (CHA), and humin (CHU) were determined. The humic acid (HA) fraction was characterized by infrared spectroscopy and the laser-induced fluorescence index (ILIF) of OM was also calculated. After six years, differences were only observed in the CHA stocks, which were highest in T1 (0.89 Mg ha-1) and T4 (1.06 Mg ha-1). The infrared spectra of HA in T1, T2 and T4 were similar to T6, with greater contribution of aliphatic organic compounds than in the other treatments. In this way, ILIF decreased in the sequence T5>T3>T4>T1>T2>T6, indicating higher OM humification in T3 and T5 and more labile OM in the other treatments. Consequently, the potential of OM quality recovery in the constructed soil was greatest in treatments T1 and T4.

Autofluorescence Study In NR2E3 p.G56R-linked Autosomal Dominant Retinitis Pigmentosa In A Single Kindred

Purpose: To date, the genotype/phenotype correlation of p.G56R-linked autosomal dominant retinitis pigmentosa (ADRP) is limited to less than 10 kindred. The purpose of this study is to report an unusual appearance of fundus autofluorescence (AF) with NR2E3 p.G56R-linked ADRP in a single kindred.Methods: Patients were enrolled among three generations in a previously unreported family. Molecular diagnosis was performed on all exons of NR2E3 and a p.G56R mutation was identified in affected family members only. Examinations included fundus photography, visual fields, optical coherence tomography, AF, near-infrared AF and ISCEV-standard electrophysiology (ERG).Results: Among 10 examined family members, 5 were affected. The youngest and oldest patients were 16 and 65 years old, respectively. Fundus examination revealed a range of retinal disorder from normal to optic nerve pallor, attenuated arterial caliber and bone spicule-like pigment deposits. In all patients, AF showed a double hyperfluorescent ring; an inner paramacular ring which extension was comparable among patients and an outer ring along the vascular arcades which extended towards periphery in older patients and became hypofluorescent. Maximal scotopic ERGs when recordable showed an increased a/b wave ratio.Conclusions: A double hyperfluorescent ring on AF is an uncommon observation and might be a specific clinical finding in NR2E3 p.G56R-linked ADRP. The consistency of that finding in all affected members of our 3-generation family confirms a previous study. Further analysis is required to determine whether AF changes are associated with particular retinal layer abnormalities.