排序方式: 共有108条查询结果,搜索用时 15 毫秒
21.
Arnaud Sartelet Tobias Stauber Wouter Coppieters Carmen F. Ludwig Corinne Fasquelle Tom Druet Zhiyan Zhang Naima Ahariz Nadine Cambisano Thomas J. Jentsch Carole Charlier 《Disease models & mechanisms》2014,7(1):119-128
Chloride-proton exchange by the lysosomal anion transporter ClC-7/Ostm1 is of pivotal importance for the physiology of lysosomes and bone resorption. Mice lacking either ClC-7 or Ostm1 develop a lysosomal storage disease and mutations in either protein have been found to underlie osteopetrosis in mice and humans. Some human disease-causing CLCN7 mutations accelerate the usually slow voltage-dependent gating of ClC-7/Ostm1. However, it has remained unclear whether the fastened kinetics is indeed causative for the disease. Here we identified and characterized a new deleterious ClC-7 mutation in Belgian Blue cattle with a severe symptomatology including perinatal lethality and in most cases gingival hamartomas. By autozygosity mapping and genome-wide sequencing we found a handful of candidate variants, including a cluster of three private SNPs causing the substitution of a conserved tyrosine in the CBS2 domain of ClC-7 by glutamine. The case for ClC-7 was strengthened by subsequent examination of affected calves that revealed severe osteopetrosis. The Y750Q mutation largely preserved the lysosomal localization and assembly of ClC-7/Ostm1, but drastically accelerated its activation by membrane depolarization. These data provide first evidence that accelerated ClC-7/Ostm1 gating per se is deleterious, highlighting a physiological importance of the slow voltage-activation of ClC-7/Ostm1 in lysosomal function and bone resorption.KEY WORDS: CLCN7, Hamartomas, Osteopetrosis, Lysosomal storage, Ion homeostasis, Belgian Blue cattle 相似文献
22.
Djemai-Zoghlache Y Isambert A Belhaneche-Bensemra N 《Journal of industrial microbiology & biotechnology》2011,38(12):1969-1978
The industrial crops of microalgae use processes calling upon the presence of parts of metal nature such as steel 316L type.
The goal of this study is to test the electrochemical behavior of this material in a marine culture of microalgae. Porphyridium purpureum was used under a photoperiod of alternation darkness/light 12/12 h, in order to apprehend the problems of biocorrosion involved
in the biofouling. The evolution of the free potential of corrosion, according to the position of the samples and for different
surface roughness, observations of the surface quality under the electron microscope with sweeping were carried out. The results
showed that, overall, the strain P. purpureum does not have a corrosive effect on the 316L. The free potential of corrosion lies between −0.307 and −0.005 V(SCE). The
adhesion of the cells seems stronger on the interface air/solid of the half-plunged sample with surface grit polished 1,000,
confirmed by the presence of biofilm on the air part. The photoperiod acts on the evolution of the generated free potential
of corrosion of the one 24-h period oscillation. Furthermore, the samples plunged horizontally lead to a stabilizing effect
on the potential of free corrosion. 相似文献
23.
Aerobic Biodegradation of DDT by Advenella Kashmirensis and Its Potential Use in Soil Bioremediation 总被引:1,自引:0,他引:1
Chiraz Abbes Ahlem Mansouri Naima Werfelli Ahmed Landoulsi 《Soil & Sediment Contamination》2018,27(6):455-468
The aim of this study was to select a bacterial strain able to degrade 1,1,1-trichloro-2,2-bis(4-chlorophenyl) ethane (DDT), and to use it for bioaugmentation in order to decontamination soil. Advenella Kashmirensis MB-PR (A. Kashmirensis MB-PR) was isolated from DDT contaminated soil, and the degradation ability of DDT by this strain in the mineral salt medium was screened by gas chromatography. The efficiency of degradation was 81% after 30 days of bacterial growth. The study of intermediary products during the degradation of DDT showed the appearance and accumulation of DDD and DDE, which emerged from the first days of the experiment. Other metabolites were detected at a lower number of chlorine atoms, such as DBH. DNA samples were isolated and screened for the linA gene, encoding dehydrochlorinase. The bioaugmentation by A. Kashmirensis MB-PR of polluted sterile soil showed that 98% of DDT disappeared after 20 days of experience. This study demonstrates the significant potential use of A. Kashmirensis MB-PR for the bioremediation of DDT in the environment. 相似文献
24.
Ekaterina Shumilina Naima Zahir Nguyen Thi Xuan Florian Lang 《Cellular physiology and biochemistry》2007,20(6):801-808
The phosphoinositide 3 (PI3) kinase plays a pivotal role in the regulation of dendritic cells (DCs), antigen-presenting cells that are able to initiate primary immune responses and to establish immunological memory. PI3 kinase is an endogenous suppressor of interleukin 12 (IL-12) production in DCs that is triggered by Toll-like receptor signaling. Inhibition of IL-12 production limits T helper 1 (Th1) polarization. On the other hand, PI3 kinase is an important regulator of various ion channels. The present study aimed to explore whether ion channels in DCs are regulated by PI3 kinase and whether they are important for DC function. To this end, DCs were isolated from murine bone marrow and ion channel activity was determined by patch clamp. As a result, DCs express voltage-gated K(+) channels (Kv), which are blocked by Stichodactyla helianthus toxin (ShK, 2.5 nM). A significant upregulation of Kv currents was observed upon maturation of DCs as induced by stimulation of the cells with lipopolysaccharide (LPS, 0.1 microg/ml, 48 h). A dramatic increase of Kv current amplitude was observed following preincubation of the cells with LY294002 (100 nM), a specific inhibitor of PI3 kinase. PI3 kinase inhibitor wortmannin (100 nM) similarly increased Kv current. LY294002 treatment was further followed by a significant increase of IL-12 production. ShK (100 nM) significantly blunted the stimulation of IL-12 release by LPS but not when the cells were first pretreated with LY294002. The observations point to Kv channel sensitive and Kv channel insensitive regulation of DC function. 相似文献
25.
Ziv Gan-Or Naima BouslamNazha Birouk Alexandra LissoubaDaniel B. Chambers Julie VérièpeAlaura Androschuk Sandra B. LaurentDaniel Rochefort Dan SpiegelmanAlexandre Dionne-Laporte Anna SzutoMeijiang Liao Denise A. FiglewiczAhmed Bouhouche Ali BenomarMohamed Yahyaoui Reda OuazzaniGrace Yoon Nicolas DupréOksana Suchowersky Francois V. BolducJ. Alex Parker Patrick A. DionPierre Drapeau Guy A. RouleauBouchra Ouled Amar Bencheikh 《American journal of human genetics》2016,98(6):1271
26.
27.
On the use of the transmission disequilibrium test to detect pseudo‐autosomal variants affecting traits with sex‐limited expression 下载免费PDF全文
Mahmoud Elansary Anneleen Stinckens Naima Ahariz Nadine Cambisano Wouter Coppieters Eli Grindflek Maren van Son Nadine Buys Michel Georges 《Animal genetics》2015,46(4):395-402
We herein describe the realization of a genome‐wide association study for scrotal hernia and cryptorchidism in Norwegian and Belgian commercial pig populations. We have used the transmission disequilibrium test to avoid spurious associations due to population stratification. By doing so, we obtained genome‐wide significant signals for both diseases with SNPs located in the pseudo‐autosomal region in the vicinity of the pseudo‐autosomal boundary. By further analyzing these signals, we demonstrate that the observed transmission disequilibria are artifactual. We determine that transmission bias at pseudo‐autosomal markers will occur (i) when analyzing traits with sex‐limited expression and (ii) when the allelic frequencies at the marker locus differ between X and Y chromosomes. We show that the bias is due to the fact that (i) sires will preferentially transmit the allele enriched on the Y (respectively X) chromosome to affected sons (respectively daughters) and (ii) dams will appear to preferentially transmit the allele enriched on the Y (respectively X) to affected sons (respectively daughters), as offspring inheriting the other allele are more likely to be non‐informative. We define the conditions to mitigate these issues, namely by (i) extracting information from maternal meiosis only and (ii) ignoring trios for which sire and dam have the same heterozygous genotype. We show that by applying these rules to scrotal hernia and cryptorchidism, the pseudo‐autosomal signals disappear, confirming their spurious nature. 相似文献
28.
Fusion to a carrier protein and a synthetic propeptide enhances E7 HPV-16 production and secretion in Lactococcus lactis 总被引:2,自引:0,他引:2
Bermúdez-Humarán LG Cortes-Perez NG Le Loir Y Gruss A Rodriguez-Padilla C Saucedo-Cardenas O Langella P Montes de Oca-Luna R 《Biotechnology progress》2003,19(3):1101-1104
An inducible system to improve and stabilize the production of an extremely labile protein (E7 antigen of human papillomavirus type 16) was developed in the food-grade bacterium Lactococcus lactis. A protein carrier, the staphylococcal nuclease Nuc, was fused either to N- or C-termini of E7 protein, and the resulting hybrid proteins were rescued from intracellular proteolysis but poorly secreted by L. lactis. A synthetic propeptide (LEISSTCDA) was then fused and significantly improved the secretion efficiency of the hybrid protein Nuc-E7 by L. lactis. 相似文献
29.
Naima Taqarort Abdelouahed Echairi Remi Chaussod Rachida Nouaim Hassan Boubaker Abdellah A. Benaoumar Elhassan Boudyach 《World journal of microbiology & biotechnology》2008,24(12):3031-3038
Epiphytic yeasts isolated from the surface of citrus fruits, harvested in several orchards in the Souss-Massa-Draa Valley,
Agadir, Morocco, were in vivo screened for antagonistic activity against Penicillium digitatum, the causal agent of green mold of citrus. From a total of 245 yeast strains assessed for their biocontrol activity against
P. digitatum, fifteen reduced the incidence of disease to less than 50%. The effectiveness of the best selected yeast strains showed that
Pichia anomala (YT73), Debaryomyces hansenii (YT22) and Hanseniaspora guilliermondii (YT13) were the most effective, with a reduction of green mold incidence from 65 to ~80%, compared to the control. The identification
of the fifteen selected yeast strains was carried out through an integrated approach including phenotypic and genotypic (sequencing
of D1/D2 domain of 26S rDNA encoding gene) methods. These 15 selected were identified as: H. guilliermondii, D. hansenii, H. uvarum and P. anomala. The study of the dynamics of two of the best strains, H. guilliermondii and D. hansenii, showed that these strains can grow rapidly, by approximately 2 log units, in citrus fruit wounds. Such rapid growth in wounds
indicates that these antagonist yeasts are excellent colonizers of citrus wounds and can thrive on citrus fruits as a substrate. 相似文献
30.
Ignatiadis M Rothé F Chaboteaux C Durbecq V Rouas G Criscitiello C Metallo J Kheddoumi N Singhal SK Michiels S Veys I Rossari J Larsimont D Carly B Pestrin M Bessi S Buxant F Liebens F Piccart M Sotiriou C 《PloS one》2011,6(1):e15624