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Stickler syndrome is an autosomal dominant disorder of the connective tissue which includes ocular and systemic manifestations. We report on a large kindred in which we were able to demonstrate very tight linkage between the disease and the type II collagen gene (COL2A1) (LOD score 3·91 at θ=0). In a family in which the father and one of his daughters were severely affected, DNA analysis from a chorionic villus sample demonstrated that the fetus possessed the normal allele of COL2A1. Thereafter a normal child was born. 相似文献
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Permanent vegetation plots were established on mixed overburden and topsoiled overburden on a lignite test pit in eastern Texas in 1982. Vegetative treatments included two grass-legume treatments [switchgrass (panicum virgattun) — sideoats grama (Bouteloua curtipendula) — subterrancan elover (Trifolitun subterranean) and litte bluestem (Schizachyriun scoparium) — sideoats grama (Bouteloua curtipendula) — Illinois hundleflower (Dessmanthus illinoiensis)] and three monocultures [Coastal bermudagrass (Cynodon dactylon), bahia grass (paspalum notatum) and yellow Indiangrass (Sorghastrum nutans)]. Water was applied to the 0.5m2 plots using a sprinklertype rainfall simulator and quality of runoff was determined for each plot. Parameters analyzed included: settleable solids, total filterable solids, sediment production, infiltration rate, nitrites, nitrates, total iron and total manganese. Topsoiling significantly increased inliltration and significantly decreased filterable sediments, sediment production and settleable solids. The hydrologie qualities of the switchgrass-sideoats grama-subterranean elover mixture coincided closely with those of the Coastal bermudagrass monoculture. 相似文献
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Municipal solid waste incinerator (MSWI) bottom ash may be used as a road construction material; it potentially contains however a sizable quantity of heavy metals, which under the effect of rainfall infiltration through the road structure can be leached out from the material and infiltrate into the underlying soil. An eco-compatibility assessment of MSWI bottom ash reuse in road construction applications necessitates examining the solubility and retention of heavy metals in road soils. This study is dedicated to Pb transfer, sorption and desorption (NEN 7341 standard test) within various soils. These experiments yield results relative to the interaction between road soils and an MSWI bottom ash leachate representative of a "fresh" product, with a high leaching potential. For the soils investigated, the sorption of lead varies between 90% and 99%. For an extraction at pH 7, Pb release is very low (<2%) for all soils, while at pH 4 leaching varies between 4% and 47%. This work shows that Pb may be fixed by some types of road soil in mostly stable forms. 相似文献
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The New Jersey Toxics Reduction Workplan for Ny-Nj Harbor: Distribution of Pcdd/Fs in Ambient Waters
As part of the NJ Toxics Reduction Workplan for NY-NJ Harbor, ambient water samples were collected at fifteen locations along
the tidal portions of the Hackensack, Passaic, Raritan, Rahway and Elizabeth Rivers, and in Newark Bay, the Arthur Kill, and
Kill van Kull. A Trace Organics Platform Sampler was used to collect a total of 75 suspended sediment phase samples between
June 2000 and May 2002. These samples were analyzed for spatial and wet vs. dry weather trends in the 17 polychlorinated dibenzo-p-dioxins
and dibenzofurans (PCDD/Fs; modified USEPA Method 1613b). Mean total PCDD/F (tPCDD/F) concentrations at the sampling locations
ranged between 3.8 and 41.5 ng/g. On average, OCDD accounted for almost 80% of the tPCDD/F concentrations; mean total [PCDD/F
– OCDD] concentrations ranged between 0.84 and 5.20 ng/g at the sampling locations. Mean 2,3,7,8-TCDD concentrations ranged
between 0.003 and 0.28 ng/g, with the highest concentrations (> 0.10 ng/g) along the tidal Passaic and lower Hackensack Rivers,
and in upper Newark Bay. Mean tPCDD/F Toxic Equivalency Quotients (TEQ) ranged between 45 and 344 TEQ pg/g, with the highest
levels found in the lower Passaic River. Toxicity was primarily driven by 2,3,7,8-TCDD concentrations in the Passaic and Hackensack
Rivers, and in Newark Bay. Examples of congener distribution patterns at some of the sampling locations are also presented. 相似文献
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To investigate the ability of elasmobranchs to distinguish between differing prey-type electric fields we examined the electroreceptive
foraging behaviour of a model species, Scyliorhinus canicula (small-spotted catshark). Catshark preferences were studied by behaviourally conditioning them to swim through narrow tunnels,
and on exit presenting them simultaneously with two different electric fields. Their subsequent choices of the following paired
options were recorded; (i) Two artificial electric fields (dipole electrodes) with different magnitude direct current (D.C.),
(ii) Two artificial electric fields, one D.C. and the other alternating current (A.C.), of the same magnitude, and (iii) similar
magnitude, natural and artificial D.C. electric fields associated with shore crabs and dipole electrodes respectively. We
found a highly significant preference for the stronger D.C. electric field and a less pronounced, but still significant, preference
for the A.C. electric field rather than the D.C. electric field. No preference was demonstrated between the artificial and
natural D.C. electric fields. The findings are discussed in relation to the animal’s diet and ecology and with regard to anthropogenic
sources of electric fields within their habitat. 相似文献
10.
Shelley C. Clarke Shelton J. Harley Simon D. Hoyle Joel S. Rice 《Conservation biology》2013,27(1):197-209
Accurate assessment of shark population status is essential for conservation but is often constrained by limited and unreliable data. To provide a basis for improved management of shark resources, we analyzed a long‐term record of species‐specific catches, sizes, and sexes of sharks collected by onboard observers in the western and central Pacific Ocean from 1995 to 2010. Using generalized linear models, we estimated population‐status indicators on the basis of catch rate and biological indicators of fishing pressure on the basis of median size to identify trends for blue (Prionace glauca), mako (Isurus spp.), oceanic whitetip (Carcharhinus longimanus), and silky (Carcharhinus falciformis) sharks. Standardized catch rates of longline fleets declined significantly for blue sharks in the North Pacific (by 5% per year [CI 2% to 8%]), for mako sharks in the North Pacific (by 7% per year [CI 3% to 11%]), and for oceanic whitetip sharks in tropical waters (by 17% per year [CI 14% to 20%]). Median lengths of silky and oceanic whitetip sharks decreased significantly in their core habitat, and almost all sampled silky sharks were immature. Our results are consistent with results of analyses of similar data sets. Combined, these results and evidence of targeted fishing for sharks in some regional fisheries heighten concerns for sustainable utilization, particularly for oceanic whitetip and North Pacific blue sharks. Regional regulations that prohibit shark finning (removal of fins and discarding of the carcass) were enacted in 2007 and are in many cases the only form of control on shark catches. However, there is little evidence of a reduction of finning in longline fisheries. In addition, silky and oceanic whitetip sharks are more frequently retained than finned, which suggests that even full implementation of and adherence to a finning prohibition may not substantially reduce mortality rates for these species. We argue that finning prohibitions divert attention from assessing whether catch levels are sustainable and that the need for management of sharks should not be addressed by measures that are simple to implement but complex to enforce and evaluate. Tendencias Poblacionales de Tiburones del Océano Pacífico y la Utilidad de Regulaciones sobre Cercenamiento de Aletas 相似文献