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An ever-expanding story of cyst formation 总被引:8,自引:0,他引:8
Gallagher AR Obermüller N Cedzich A Gretz N Witzgall R 《Cell and tissue research》2000,300(3):361-371
Autosomal-dominant polycystic kidney disease represents one of the most common monogenetic human disorders. The cloning of the PKD1 and PKD2 genes, which are mutated in far more than 90% of the patients affected by this disease, has generated high hopes for a quick understanding of the pathogenesis of cyst formation. However, these expectations have not yet been fulfilled, since the function of both polycystin-1 and polycystin-2, the two proteins encoded by PKD1 and PKD2, still remains a puzzle. In this review, we will highlight some of the characteristics of polycystic kidney disease, briefly touch on polycystin-1, and then go on to describe recent results of experiments with polycystin-2, since the latter is the major focus of our work. We will discuss new evidence which suggests that autosomal-dominant polycystic kidney disease actually behaves recessively on a cellular level. Finally, a model will be presented that tries to explain the available data. 相似文献
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The effects of phosphate (P) limitation, varying salinity (5–65 psu), and solid media growth conditions on the polysaccharides produced by the model diatom, Phaeodactylum tricornutum Bohlin were determined. Sequential extraction was used to separate polymers into colloidal (CL), colloidal extracellular polymeric substances (cEPS), hot water soluble (HW), hot bicarbonate soluble (HB), and hot alkali (HA) soluble fractions. Media‐soluble polymers (CL and cEPS) were enriched in 4‐linked mannosyl, glucosyl, and galactosyl residues as well as terminal and 3‐linked xylosyl residues, whereas HW polymers consisted mainly of 3‐linked glucosyl as well as terminal and 2,4‐linked glucuronosyl residues. The HB fraction was enriched in terminal and 2‐linked rhamnosyl residues derived from the mucilage coating solubilized by this treatment. Hot alkali treatment resulted in the complete dissolution of the frustule releasing 2,3‐ and 3‐linked mannosyl residues. The fusiform morphotype predominated in standard and P‐limited cultures and cultures subjected to salinity variations, but growth on solid media resulted in an enrichment of the oval morphotype. The proportion and linkages of 15 residues, including neutral, uronic acid, and O‐methylated sugars, varied with environmental conditions. P limitation and salinity changes resulted in 1.5‐ to 2.5–fold increase in carbohydrate production, with enrichment of highly branched/substituted and terminal rhamnose, xylose, and fucose as well as O‐methylated sugars, uronic acids, and sulfate. The increased deoxy‐ and O‐methylated sugar content under unfavorable environments enhances the hydrophobicity of the polymers, whereas the anionic components may play important roles in ionic cross‐linking, suggesting that these changes could ameliorate the effects of salinity or P‐stress and that these altered polysaccharide characteristics may be useful as bioindicators for environmental stress. 相似文献
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A technique is described for the intermittent collection of blood from the rat tail. By using commonly available equipment, blood samples can easily be obtained from rats without the need for anaesthesia. The development of this technique makes the rat more readily available as an animal model for repeated withdrawals of small blood samples for pharmacokinetic or bioavailability evaluations. 相似文献
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Schalk Robert Braun Frank Frank Rudolf Rädle Matthias Gretz Norbert Methner Frank-Jürgen Beuermann Thomas 《Bioprocess and biosystems engineering》2017,40(10):1519-1527
Bioprocess and Biosystems Engineering - The monitoring of microbiological processes using Raman spectroscopy has gained in importance over the past few years. Commercial Raman spectroscopic... 相似文献
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Summary Acidosis is a common finding in uremia. We analysed the effect of changes in acid-base balance in 42 Sprague-Dawley rats having longstanding uremia (> 3 mon). The rats were treated with CaCO3 or CaCl2 in a random cross-over trial after being divided into two dietary groups (8% or 18% protein). At the end of each observation period parameters were assessed. The main finding was that plasma amino and keto acid concentrations varied significantly with the acid-base balance and the protein intake. Furthermore a considerable degree of catabolism and anorexia was observed. In addition other confounding variables were observed, e.g. increased corticosterone excretion, electrolyte losses, and proteinuria. Our data underline that plasma amino acid concentrations in uremic rats are highly dependent on acid-base balance and overall protein intake. 相似文献