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  • 1
    ISSN: 1432-0533
    Keywords: Key words CADASIL ; Electron microscopy ; Granular osmiophilic material ; αB crystallin ; Heat ; shock proteins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a non-arterio-atherosclerotic, non-amyloidotic arteriopathy affecting preferentially the small arteries and arterioles of the brain. The morphologic hallmark is the presence of a characteristic granular alteration of the arterial media that ultrastructurally corresponds to the accumulation of electron-dense material surrounding the smooth muscle cells. Although the presence of this granular osmiophilic material (GOM) was originally described as limited to brain vessels, identical electron microscopic findings have been demonstrated in the media of peripheral tissue arteries, allowing for a pathologic diagnosis of the disease by a simple skin, muscle or nerve biopsy. We report some atypical features identified in our CADASIL patients that broaden the phenotypic expression of this disease. Firstly, we identified a cortical infarct in an otherwise typical CADASIL patient. Secondly, we observed GOM in skin arteries of a 30-year-old man with hemiplegic migraine, the son of a woman who had died with CADASIL. This confirms that it may be possible to diagnose the disease at a preclinical stage by the ultrastructural evaluation of peripheral tissue biopsy material, particularly for individuals for whom there is a supporting family history. Thirdly, ultrastructural examination of the skin, and subcutaneous and striated muscle of an unrelated and apparently sporadic patient with neuropathologic and neuroradiologic evidence of CADASIL in meningeal and cerebral vessels failed to reveal diagnostic lesions in peripheral arteries. Thus, the possibility of a false-negative pathologic diagnosis in patients with a clinicoradiologic diagnosis of CADASIL, if one relies solely on a peripheral tissue biopsy, does exist. Additionally, we have identified heat shock proteins (Hsp70 and αB crystallin) and ubiquitin in the vascular myocytes of affected arteries. αB crystallin also seemed to be deposited extracellularly, which suggests that GOM also might be immunoreactive for αB crystallin.
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  • 2
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Basaloid proliferations overlying dermatofibromas which morphologically resemble superficial basal cell carcinomas have been interpreted as both reactive/regressive and frankly malignant. Metallothioneins (MTs) are low-molecular-weight proteins with a selective binding affinity for heavy metal ions. MTs has been proposed to represent a biological marker of carcinogenesis and, in a variety of human tumours, a correlation between immunohistochemically overexpresstion of MT and aggressive clinical behaviour has been shown. In order to clarify the nature of basaloid proliferations overlying dermatofibromas, we examined, immunohistochemically, 10 dermatofibromas with overlying simple hyperplasia, 16 dermatofibromas with overlying basaloid proliferation, and 35 basal cell carcinomas, for expression of MT.In normal epidermis, the basal keratinocytes showed cytoplasmatlc MT immunoreactivity. The staining intensity was stronger in the basal cells of the rete ridges, an observation which is in accordance with the high proportion of S-phase cells in this area. Simple hyperplasia showed the same MT expression pattern as normal epidermis. Basaloid proliferations stained like superficial and nodular basal cell carcinomas. Of nodular basal cell carcinomas, 92% (12 of 13) showed decreased/absent MT immunoreactivity, while 86% (six of seven) of infiltrating/morphoea-like basal cell carcinomas showed overexpression of MT (P = 0.001, Fisher's exact test). The results demonstrate that MT overexpression in basal cell carcinomas is correlated with infiltrative growth pattern. The similar expression of MT in basaloid proliferations and ‘non-infiltrating’ basal cell carcinomas suggests that these lesions share a common change in metabolism and/or differentiation.
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  • 3
    ISSN: 1433-7347
    Keywords: Key words Anterior cruciate ; ligament ; Isolated partial rupture ; Knee laxity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine , Sports Science
    Notes: Abstract The majority of previous studies on partial ruptures of the anterior cruciate ligament (ACL) include a relatively large proportion of knees with associated intra-articular injury or collateral ligament tear that contributes to an increase in the symptoms of instability and further deterioration of knee function. In the present study only patients with isolated, partial ruptures of the ACL were evaluated. Fifty-six patients with one injured knee were examined after a median of 5.3 (range 2.0–12.7) years using the IKDC evaluation form, Lysholm knee function score and Tegner activity score. Of the 56 knees, 6 underwent autologous reconstruction due to early progression to complete rupture. Of 34 knees evaluated for laxity, 25 had a negative Lachman test and 7 a positive (+) Lachman. In 2 knees a Lachman ++ result and a positive pivot shift were found. With instrumented laxity testing 24 knees had 2 mm or less difference in laxity compared with the contralateral uninjured knee. The largest side-to-side difference in knee laxity was 4.5 mm. Lysholm score was median 86 (range 52–100) points, and 62% had good or excellent knee function. A significant decline in activity was seen. Only 10 patients (30%) resumed their preinjury activities. We find that the majority of patients with an isolated, partial rupture of the ACL have an acceptable knee function and a stable knee after a median 5 years follow-up. There is, however, a marked reduction in activity.
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  • 4
    ISSN: 1399-0047
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: Dihydroorotate dehydrogenases are flavin-containing enzymes which catalyze the conversion of (S)-dihydroorotate to orotate. Dihydroorotate dehydrogenase B (DHODB) from Lactococcus lactis is a heterotetramer containing two subunits of the protein encoded by the pyrDb gene (PyrDB) and two subunits of the protein encoded by the pyrK gene (PyrK). In addition, DHODB contains two molecules of flavin mononucleotide, two molecules of flavin adenine dinucleotide and two [2Fe–2S] iron–sulfur clusters as tightly bound cofactors. Yellow crystals of this enzyme have been grown using the hanging-drop vapour-diffusion technique from solutions of 2.5 M ammonium sulfate and 0.1 M sodium acetate, pH 4.6. The crystals have been shown to contain both the PyrDB and the PyrK subunits and fluorescence measurements indicate that the two different subunits interact very closely with each other in the active-site region. Native data sets have been collected to 2.6 Å with a conventional X-ray source and to 2.2 Å using synchrotron radiation. The crystals are rhombohedral, space group R32, with correspondin8 hexagonal unit-cell dimensions a = b = 202.3 and c = 81.0 Å. The asymmetric unit in the crystal contains one PyrDB subunit and one PyrK subunit, which suggests that the two halves of the heterotetramer are related by a crystallographic twofold axis.
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Plant, cell & environment 20 (1997), S. 0 
    ISSN: 1365-3040
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: Mentha aquatica L. was grown at different nutrient availabilities in water and in air at 60% RH. The plants were kept at 600 mmol m−3 free CO2 dissolved in water (40 times air equilibrium) and at 30 mmol m−3 CO2 in air to ensure CO2 saturation of growth in both environments. We quantified the transpiration-independent water transport from root to shoot in submerged plants relative to the transpiration stream in emergent plants and tested the importance of transpiration in sustaining nutrient flux and shoot growth. The acropetal water flow was substantial in submerged Mentha aquatica, reaching 14% of the transpiration stream in emergent plants. The transpiration-independent mass flow of water from the roots, measured by means of tritiated water, was diverted to leaves and adventitious shoots in active growth. The plants grew well and at the same rates in water and air, but nutrient fluxes to the shoot were greater in plants grown in air than in those that were submerged when they were rooted in fertile sediments. Restricted O2 supply to the roots of submerged plants may account for the smaller nutrient concentrations, though these exceeded the levels required to saturate growth. In hydroponics, the root medium was aerated and circulated between submerged and emergent plants to minimize differences in medium chemistry, and here the two growth forms behaved similarly and could fully exploit nutrient enrichment. It is concluded that the lack of transpiration from leaf surfaces in a vapour-saturated atmosphere, or under water, is not likely to constrain the transfer of nutrients from root to shoot in herbaceous plants. Nutrient deficiency under these environmental conditions is more likely to derive from restricted development and function of the roots in waterlogged anoxic soils or from low porewater concentrations of nutrients.
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  • 6
    ISSN: 1573-5117
    Keywords: Corophium ; Hydrobia ; intertidal mud flats ; microphallid trematodes ; phenology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract The phenology of microphallid trematodes within their intermediate hostpopulations has been studied on an intertidal mud flat. The parasites usethe mud snail Hydrobia ulvae and the infaunal amphipod Corophium volutatoras first and secondary intermediate host, respectively. Migratory shorebirdsact as final hosts. Our results show a general trend of decline in thedensity of infected intermediate hosts during both spring and autumn, whichcould mainly be ascribed to shorebird predation. During summer the densityof both infected snails and infected amphipods increased considerably, witha culmination in June within the snail population (1000 infectedm-2 and in August within the amphipod population (40 000infected m-2. This time lag in parasite occurrence could berelated to (1) the development time of larval trematodes within the snails,(2) higher ambient temperatures in late summer increasing parasitetransmission between snails and amphipods during this period, and (3) ageneral increase in the Corophium population during late summer. Fromsamples collected between 1990 and 1995 it is shown that microphallidtrematodes occasionally may give rise to mass mortality in the amphipodpopulation. The prerequisites for such an event are a high parasiteprevalence within the first intermediate host population and unusually highambient temperatures, facilitating parasite transmission to the secondaryintermediate host, C. volutator.
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  • 7
    ISSN: 1089-7550
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Microfabricated field emitter arrays (FEAs) can provide the very high electron current densities required for rf amplifier applications, typically on the order of 100 A/cm2. Determining the dependence of emission current on gate voltage is important for the prediction of emitter performance for device applications. Field emitters use high applied fields to extract current, and therefore, unlike thermionic emitters, the current densities can exceed 103 A/cm2 when averaged over an array. At such high current densities, space charge effects (i.e., the influence of charge between cathode and collector on emission) affect the emission process or initiate conditions which can lead to failure mechanisms for field emitters. A simple model of a field emitter will be used to calculate the one-dimensional space charge effects on the emission characteristics by examining two components: charge between the gate and anode, which leads to Child's law, and charge within the FEA unit cell, which gives rise to a field suppression effect which can exist for a single field emitter. The predictions of the analytical model are compared with recent experimental measurements designed to assess space charge effects and predict the onset of gate current. It is shown that negative convexity on a Fowler–Nordheim plot of Ianode(Vgate) data can be explained in terms of field depression at the emitter tip in addition to reflection of electrons by a virtual cathode created when the anode field is insufficient to extract all of the current; in particular, the effects present within the unit cell constitute a newly described effect.
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