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2.
Locus Heterogeneity for Waardenburg Syndrome is Predictive of Clinical Subtypes 总被引:5,自引:4,他引:1
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Lindsay A. Farrer Kathleen S. Arnos James H. Asher Clinton T. Baldwin Scott R. Diehl Thomas B. Friedman Jacquie Greenberg Kenneth M. Grundfast Christopher Hoth Anil K. Lalwani Barbara Landa Kate Leverton Aubrey Milunsky Robert Morell Walter E. Nance Valerie Newton Rajkumar Ramesar Valluri S. Rao Jennifer E. Reynolds Theresa B. San Agustin Edward R. Wilcox Ingrid Winship Andrew P. Read 《American journal of human genetics》1994,55(4):728-737
Waardenburg syndrome (WS) is a dominantly inherited and clinically variable syndrome of deafness, pigmentary changes, and distinctive facial features. Clinically, WS type I (WS1) is differentiated from WS type II (WS2) by the high frequency of dystopia canthorum in the family. In some families, WS is caused by mutations in the PAX3 gene on chromosome 2q. We have typed microsatellite markers within and flanking PAX3 in 41 WS1 kindreds and 26 WS2 kindreds in order to estimate the proportion of families with probable mutations in PAX3 and to study the relationship between phenotypic and genotypic heterogeneity. Evaluation of heterogeneity in location scores obtained by multilocus analysis indicated that WS is linked to PAX3 in 60% of all WS families and in 100% of WS1 families. None of the WS2 families were linked. In those families in which equivocal lod scores (between −2 and +1) were found, PAX3 mutations have been identified in 5 of the 15 WS1 families but in none of the 4 WS2 families. Although preliminary studies do not suggest any association between the phenotype and the molecular pathology in 20 families with known PAX3 mutations and in four patients with chromosomal abnormalities in the vicinity of PAX3, the presence of dystopia in multiple family members is a reliable indicator for identifying families likely to have a defect in PAX3. 相似文献
3.
Abstract The thymus of the sole Solea solea contained lymphoblasts and thymocytes within a network of pale and dark epithelial cells. The pale cells were characterized by tonofilaments and desmosomes and some embraced rodlet cells within their cytoplasmic processes. The dark epithelial cells had numerous electron-dense inclusions and electron-lucent vacuoles. Lymphocytes were closely associated with the plasma membrane of both types of epithelial cells and with macrophages. Breakdown of effete lymphocytes appeared to be the main function of the macrophages. Some macrophages were multinucleated. Those containing melanin granules associated with phagosomes were classified as melanomacrophages. Pigment cells including melanophores and guanophores were present along the connective tissue trabeculae and surrounding the blood vessels. A few plasma cells and mucous cells were present. 相似文献
4.
Between June 1986 and October 1992, in 3 periods totaling 12 months, we studied social organization of pigtailed langurs (Simias concolor)in the Pagai Islands, Indonesia. With one possible exception, all of 20 family groups contained only 1 adult male each. Nine
of them contained only 1 adult female,and 11 contained ≥2 adult females. Mean group size is 4.1 individuals. Home ranges varied from 7 to 20 ha, and population
density averages 21 animals/km
2
. Simias concoloris sexually dimorphic⤖n a museum sample males average 29% heavier than females, and their canine teeth are on average nearly
twice the length of female canines. Combined data from several studies indicate that 60% of S. concolorgroups contain an adult pair plus young,and 40% are multifemale groupings. We sggest that any 1-male mating system that comprises a mixture of 1-female and multifemale
groups in the same population should be termed monandry. 相似文献
5.
Summary The effect of alkaline treatment on nucleic acid content and in vitro dry matter digestibility of C. utilis NRRL Y-660 has been evaluated using sodium and ammonium hydroxides for the treatment and finding the former preferable. Total dry matter and true protein recovery are also reported. 相似文献
6.
Purification and structure of caltrin-like proteins from seminal vesicle of the guinea pig 总被引:1,自引:0,他引:1
Two different small proteins that cross-react with the antiserum against bovine caltrin (calcium transport inhibitor) have been purified from the seminal vesicle contents of the guinea pig. The primary structure and some molecular characteristics of the pure proteins are reported. The two proteins interact with concanavalin A indicating the presence of carbohydrates in their molecules. Chemical deglycosylation with trifluoromethanesulfonic acid, after reduction and carboxymethylation, results in complete loss of affinity for the lectin. Removal of sugar components from the structure destroys the ability of caltrin-like proteins to react with antibodies to bovine caltrin. The protein moving faster on polyacrylamide gel electrophoresis is designated guinea pig caltrin I, the other is II. They contain 45 and 55 amino acids, and the molecular weights of the peptide portions are 5082 and 6255, respectively. Although they have entirely different amino acid sequences, they share some common features: recognition by rabbit antibodies to bovine caltrin, the predominance of basic residues and the presence of 3 cysteine residues in fraction I and 8 in fraction II. The proteins have pI values of 9.5 and 10.2, respectively, which are consistent with the amino acid composition. The two pure fractions are approximately equally effective, on a weight basis, as inhibitors of 45Ca2+ uptake by guinea pig spermatozoa. The data presented reinforce the hypothesis that caltrin-like proteins are responsible for the previously reported (Coronel, C.E., San Agustin, J., and Lardy, H.A. (1988) Biol. Reprod. 38, 713-722), calcium-transport inhibitor activity detected in reproductive tract fluid from adult male guinea pigs. 相似文献
7.
Agustin Luz-Madrigal Alexander Asanov Aldo R. Camacho-Zarco Alicia Sampieri Luis Vaca 《Journal of virology》2013,87(21):11894-11907
Baculoviridae is a large family of double-stranded DNA viruses that selectively infect insects. Autographa californica multiple nucleopolyhedrovirus (AcMNPV) is the best-studied baculovirus from the family. Many studies over the last several years have shown that AcMNPV can enter a wide variety of mammalian cells and deliver genetic material for foreign gene expression. While most animal viruses studied so far have developed sophisticated mechanisms to selectively infect specific cells and tissues in an organism, AcMNPV can penetrate and deliver foreign genes into most cells studied to this date. The details about the mechanisms of internalization have been partially described. In the present study, we have identified a cholesterol recognition amino acid consensus (CRAC) domain present in the AcMNPV envelope fusion protein GP64. We demonstrated the association of a CRAC domain with cholesterol, which is important to facilitate the anchoring of the virus at the mammalian cell membrane. Furthermore, this initial anchoring favors AcMNPV endocytosis via a dynamin- and clathrin-dependent mechanism. Under these conditions, efficient baculovirus-driven gene expression is obtained. In contrast, when cholesterol is reduced from the plasma membrane, AcMNPV enters the cell via a dynamin- and clathrin-independent mechanism. The result of using this alternative internalization pathway is a reduced level of baculovirus-driven gene expression. This study is the first to document the importance of a novel CRAC domain in GP64 and its role in modulating gene delivery in AcMNPV. 相似文献
8.
François Binet Gaëlle Mawambo Nicholas Sitaras Nicolas Tetreault Eric Lapalme Sandra Favret Agustin Cerani Dominique Leboeuf Sophie Tremblay Flavio Rezende Aimee M. Juan Andreas Stahl Jean-Sebastien Joyal Éric Milot Randal J. Kaufman Martin Guimond Timothy E. Kennedy Przemyslaw Sapieha 《Cell metabolism》2013,17(3):353-371
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9.
Olga Malinkiewicz Thais Grancha Agustin Molina‐Ontoria Alejandra Soriano Hicham Brine Henk J. Bolink 《Liver Transplantation》2013,3(4):472-477
Simple bilayer solar cells, using commercially available cationic cyanine dyes as donors and evaporated C60 layer as an acceptor are prepared. Cyanine dyes with absorption maxima of 578, 615 and 697 nm having either perchlorate or hexafluorophosphate counter‐ions are evaluated. The perchlorate dye leads to cells with S‐shape current‐voltage curves; only the dyes with the hexafluorophosphate counter‐ions lead to efficient solar cells. When the wide bandgap dyes are employed, S‐shape current‐voltage curves are obtained when the conductive polymer PEDOT:PSS is used as hole transport layer. Substitution of PEDOT:PSS with MoO3 leads to cells with more rectangular current–voltage curves and high fill factors. Additionally, the cells using the MoO3 layer for hole extraction lead to high open circuit voltages of 0.9 V. In the case that a low bandgap hexafluorophosphate dye is used with the HOMO above that of the PEDOT:PSS the cell performance is independent on the type of hole transport layer employed. Using this approach, bilayer solar cells are obtained with power efficiencies ranging from 1.8 to 2.9% depending on the particular dye employed. These are impressive numbers for bilayer solar cell that are partially solution processed in ambient conditions. 相似文献
10.
Dietary management of the human gut microbiota towards a more beneficial composition is one approach that may improve host health. To date, a large number of human intervention studies have demonstrated that dietary consumption of certain food products can result in significant changes in the composition of the gut microbiota i.e. the prebiotic concept. Thus the prebiotic effect is now established as a dietary approach to increase beneficial gut bacteria and it has been associated with modulation of health biomarkers and modulation of the immune system. Promitor™ Soluble Corn Fibre (SCF) is a well-known maize-derived source of dietary fibre with potential selective fermentation properties. Our aim was to determine the optimum prebiotic dose of tolerance, desired changes to microbiota and fermentation of SCF in healthy adult subjects. A double-blind, randomised, parallel study was completed where volunteers (n = 8/treatment group) consumed 8, 14 or 21 g from SCF (6, 12 and 18 g/fibre delivered respectively) over 14-d. Over the range of doses studied, SCF was well tolerated Numbers of bifidobacteria were significantly higher for the 6 g/fibre/day compared to 12g and 18g/fibre delivered/day (mean 9.25 and 9.73 Log10 cells/g fresh faeces in the pre-treatment and treatment periods respectively). Such a numerical change of 0.5 Log10 bifidobacteria/g fresh faeces is consistent with those changes observed for inulin-type fructans, which are recognised prebiotics. A possible prebiotic effect of SCF was therefore demonstrated by its stimulation of bifidobacteria numbers in the overall gut microbiota during a short-term intervention. 相似文献