Cerebrohepatorenal malformation is a rare familial disorder characterized by typical renal lesions combined with Dandy-Walker malformation, and congenital hepatic fibrosis. In this case report, a male premie with the diagnosis of cerebrorenal syndrome or so called Goldston syndrome is presented. Besides the rarity of this syndrome, this case is the second reported patient diagnosed prenatally. 相似文献
Summary Using the immunoperoxidase technique in conjunction with specific antisera to -atrial natriuretic polypeptide (-ANP), it was shown that immunoreactive cell bodies and varicose fibers are widely distributed throughout the rat brain. The highest concentrations of -ANP-containing neuronal cell bodies and fibers were found in the hypothalamus and septum. This result confirms the radioimmunological determination of -ANP immunoreactivity in the rat brain. 相似文献
A new spin-label, 4-(L-glutamo)-4'-[(1-oxy-2,2,5,5-tetramethyl-3L-pyrrolidinyl )amino]-3, 3'-dinitrodiphenyl sulfone, is shown to bind to one high-affinity binding site on bovine serum albumin (K = 5 X 10(4) M-1, n = 1). Analysis of the binding of the spin-label to the amino-terminal half (peptic fragment PB) and the carboxy-terminal half (peptic fragment PA) of BSA, and their complex (PA-PB), indicates that the spin-label binds to a long-chain fatty acid binding site located on PB. The usefulness of the novel specificity of the spin-label in characterizing this binding site is discussed. 相似文献
We have determined the nucleotide sequence coding for the chicken brain alpha-spectrin. It is derived both from the cDNA and genomic sequences, comprises the entire coding frame, 5' and 3' untranslated sequences, and terminates in the poly(A)-tail. The deduced amino acid sequence was used to map the domain structure of the protein. The alpha-chain of brain spectrin contains 22 segments of which 20 correspond to the repeat of the human erythrocyte spectrin (Speicher, D. W., and V. T. Marchesi. 1984. Nature (Lond.). 311:177-180.), typically made of 106 residues. These homologous segments probably account for the flexible, rod-like structure of spectrin. Secondary structure prediction suggests predominantly alpha-helical structure for the entire chain. Parts of the primary structure are excluded from the repetitive pattern and they reside in the middle part of the sequence and in its COOH terminus. Search for homology in other proteins showed the presence of the following distinct structures in these nonrepetitive regions: (a) the COOH-terminal part of the molecule that shows homology with alpha-actinin, (b) two typical EF-hand (i.e., Ca2+-binding) structures in this region, (c) a sequence close to the EF-hand that fulfills the criteria for a calmodulin-binding site, and (d) a domain in the middle of the sequence that is homologous to a NH2-terminal segment of several src-tyrosine kinases and to a domain of phospholipase C. These regions are good candidates to carry some established as well as some yet unestablished functions of spectrin. Comparative analysis showed that alpha-spectrin is well conserved across the species boundaries from Xenopus to man, and that the human erythrocyte alpha-spectrin is divergent from the other spectrins. 相似文献
NinePseudomonas strains were selected by their high copper tolerance from a population of bacteria isolated from heavy-metal polluted zones. Copper resistance (Cur) was inducible by previous exposure of cultures to subinhibitory amounts of copper sulfate. All nine strains possessed large plasmids, but transformation and curing results suggest that Cur is conferred by chromosomal genes. Plasmid-lessPseudomonas aeruginosa PAO-derived strains showed the same level of Cur as environmental isolates and their resistance to copper was also inducible. Total DNA from the environmentalPseudomonas, as well as fromP. aeruginosa PAO strains, showed homology to a CurP. syringae cop probe at low-stringency conditions but failed to hybridize at high-stringency conditions. 相似文献
Summary Glomus (Type I) cells of the carotid body of adult rats were studied electron microscopically after fixation with potassium permanganate or with glutaraldehyde and osmium tetroxide. Two permanganate fixation methods (using Krebs-Ringer-glucose, pH 7.0, or acetate buffer, pH 5.0) were compared. Numerous dense-cored vesicles were observed only in about one tenth of the glomus cells when neutral permanganate was used for fixation, although all glomus cells showed such vesicles after fixation with glutaraldehyde and osmium tetroxide. Numerous vesicles with a dense core were observed in about one third of the cells after fixation with acid potassium permanganate. With this fixation, small dense-cored vesicles similar to those in adrenergic nerve terminals were occasionally seen in the cytoplasm of glomus cells. It is tentatively concluded that the amine-storing vesicles of the carotid body are different from those in the small intensely fluorescent (SIF) cells and those in adrenergic nerve terminals. 相似文献
Recent researches suggest that functional diversity represents the response of communities to environmental alterations better than taxonomic diversity. However, there is scarce information about how the functional diversity of freshwater fishes is affected by habitat type and the dominance of non-native species. To address this question, we analysed a large database containing 15 morpho-functional traits of 61 fish species from the Pannon Biogeographic region (Hungary). Based on a fish faunistic list and relative abundance of taxa, we quantified the taxonomic and functional diversity of riverine communities for?>?700 sites of six habitat types. We asked how non-native fishes affected the taxonomic and functional diversity in different river types and at the local scale (i.e. at the site level), and how the diversity measures of native fauna elements changes along the invasion gradient. Our results showed that both functional and taxonomic richness increases with habitat complexity, from small headwater streams to large rivers. Therefore taxonomic diversity served as a good proxy for functional diversity along the environmental gradient of river types. Non-natives showed considerable functional diversity relative to their species number in each habitat type. Diversity values of native fauna elements initially increased, and then showed a major decrease along the invasion gradient. River type-specific evaluations highlighted the importance of considering the proliferation of invasive species based on both taxonomic and functional diversity indices. We argue that type-specific action plans are needed in conservation management to preserve the taxonomic and functional diversity of native fishes in Hungary, but also elsewhere.