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All affected patients in four families with autosomal dominant familial renal tubular acidosis (dRTA) were heterozygous for mutations in their red cell HCO3-/Cl- exchanger, band 3 (AE1, SLC4A1) genes, and these mutations were not found in any of the nine normal family members studied. The mutation Arg589--> His was present in two families, while Arg589--> Cys and Ser613--> Phe changes were found in the other families. Linkage studies confirmed the co-segregation of the disease with a genetic marker close to AE1. The affected individuals with the Arg589 mutations had reduced red cell sulfate transport and altered glycosylation of the red cell band 3 N-glycan chain. The red cells of individuals with the Ser613--> Phe mutation had markedly increased red cell sulfate transport but almost normal red cell iodide transport. The erythroid and kidney isoforms of the mutant band 3 proteins were expressed in Xenopus oocytes and all showed significant chloride transport activity. We conclude that dominantly inherited dRTA is associated with mutations in band 3; but both the disease and its autosomal dominant inheritance are not related simply to the anion transport activity of the mutant proteins.  相似文献   
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This paper considers the non-linear stress-strain characteristics of a particulate material using the classical elasticity concepts of modulus of elasticity and Poisson's ratio and shows that these terms are stress-dependent. In addition to the first loading of the material a number of unloading/reloading cycles are carried out under conditions representative of practical loading cases. Finally, recommendations are made regarding the analysis of problems involving particulate materials subjected to three-dimensional stress and strain.  相似文献   
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Ray‐traced global illumination (GI) is becoming widespread in production rendering but incoherent secondary ray traversal limits practical rendering to scenes that fit in memory. Incoherent shading also leads to intractable performance with production‐scale textures forcing renderers to resort to caching of irradiance, radiosity, and other values to amortize expensive shading. Unfortunately, such caching strategies complicate artist workflow, are difficult to parallelize effectively, and contend for precious memory. Worse, these caches involve approximations that compromise quality. In this paper, we introduce a novel path‐tracing framework that avoids these tradeoffs. We sort large, potentially out‐of‐core ray batches to ensure coherence of ray traversal. We then defer shading of ray hits until we have sorted them, achieving perfectly coherent shading and avoiding the need for shading caches.  相似文献   
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Abstract

This paper is a review of the literature concerning the preparation of [60]fullerene amino acid and peptide derivatives. The structure and applications of these derivatives to the biological and Material sciences is also presented.  相似文献   
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Fourier transform infrared spectroscopy (FTIR), scanning electron microscopy (SEM) and X-ray photoelectron spectroscopy (XPS) were employed to analyze the surfaces of PVC films with poor printability. FTIR indicated that, independent of formulation, the migrating species were metal carboxylates. Different techniques penetrate the surface to different depths allowing examination of a range of surface layers. XPS indicated that, in the worst case, the upper surface layer was comprised almost exclusively of a mixed metal carboxylate. Atom percentages obtained by XPS indicated hydrolysis of the mixed metal carboxylate at the vinyl surface.  相似文献   
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A selective mono-N-arylation strategy of amidines under Chan-Lam conditions is described. During the reaction optimization phase, the isolation of a mononuclear Cu(II) complex provided unique mechanistic insight into the operation of Chan-Lam mono-N-arylation. The scope of the process is demonstrated, and then applied to access the first mono-N-arylated analogues of pentamidine. Sub-micromolar activity against kinetoplastid parasites was observed for several analogues with no cross-resistance in pentamidine and diminazene-resistant trypanosome strains and against Leishmania mexicana. A fluorescent mono-N-arylated pentamidine analogue revealed rapid cellular uptake, accumulating in parasite nuclei and the kinetoplasts. The DNA binding capability of the mono-N-arylated pentamidine series was confirmed by UV-melt measurements using AT-rich DNA. This work highlights the potential to use Chan-Lam mono-N-arylation to develop therapeutic leads against diamidine-resistant trypanosomiasis and leishmaniasis.  相似文献   
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