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排序方式: 共有114条查询结果,搜索用时 171 毫秒
11.
Valsecchi F Monge C Forkink M de Groof AJ Benard G Rossignol R Swarts HG van Emst-de Vries SE Rodenburg RJ Calvaruso MA Nijtmans LG Heeman B Roestenberg P Wieringa B Smeitink JA Koopman WJ Willems PH 《Biochimica et biophysica acta》2012,1817(10):1925-1936
Human mitochondrial complex I (CI) deficiency is associated with progressive neurological disorders. To better understand the CI pathomechanism, we here studied how deletion of the CI gene NDUFS4 affects cell metabolism. To this end we compared immortalized mouse embryonic fibroblasts (MEFs) derived from wildtype (wt) and whole-body NDUFS4 knockout (KO) mice. Mitochondria from KO cells lacked the NDUFS4 protein and mitoplasts displayed virtually no CI activity, moderately reduced CII, CIII and CIV activities and normal citrate synthase and CV (F(o)F(1)-ATPase) activity. Native electrophoresis of KO cell mitochondrial fractions revealed two distinct CI subcomplexes of ~830kDa (enzymatically inactive) and ~200kDa (active). The level of fully-assembled CII-CV was not affected by NDUFS4 gene deletion. KO cells exhibited a moderately reduced maximal and routine O(2) consumption, which was fully inhibited by acute application of the CI inhibitor rotenone. The aberrant CI assembly and reduced O(2) consumption in KO cells were fully normalized by NDUFS4 gene complementation. Cellular [NAD(+)]/[NADH] ratio, lactate production and mitochondrial tetramethyl rhodamine methyl ester (TMRM) accumulation were slightly increased in KO cells. In contrast, NDUFS4 gene deletion did not detectably alter [NADP(+)]/[NADPH] ratio, cellular glucose consumption, the protein levels of hexokinases (I and II) and phosphorylated pyruvate dehydrogenase (P-PDH), total cellular adenosine triphosphate (ATP) level, free cytosolic [ATP], cell growth rate, and reactive oxygen species (ROS) levels. We conclude that the NDUFS4 subunit is of key importance in CI stabilization and that, due to the metabolic properties of the immortalized MEFs, NDUFS4 gene deletion has only modest effects at the live cell level. This article is part of a special issue entitled: 17th European Bioenergetics Conference (EBEC 2012). 相似文献
12.
Mayr JA Haack TB Graf E Zimmermann FA Wieland T Haberberger B Superti-Furga A Kirschner J Steinmann B Baumgartner MR Moroni I Lamantea E Zeviani M Rodenburg RJ Smeitink J Strom TM Meitinger T Sperl W Prokisch H 《American journal of human genetics》2012,90(2):314-320
Exome sequencing of an individual with congenital cataracts, hypertrophic cardiomyopathy, skeletal myopathy, and lactic acidosis, all typical symptoms of Sengers syndrome, discovered two nonsense mutations in the gene encoding mitochondrial acylglycerol kinase (AGK). Mutation screening of AGK in further individuals with congenital cataracts and cardiomyopathy identified numerous loss-of-function mutations in an additional eight families, confirming the causal nature of AGK deficiency in Sengers syndrome. The loss of AGK led to a decrease of the adenine nucleotide translocator in the inner mitochondrial membrane in muscle, consistent with a role of AGK in driving the assembly of the translocator as a result of its effects on phospholipid metabolism in mitochondria. 相似文献
13.
Gerwin D. Rodenburg Elisabeth A. M. Sanders Elske J. M. van Gils Reinier H. Veenhoven Tomasz Zborowski Germie P. J. M. van den Dobbelsteen Andries C. Bloem Guy A. M. Berbers Debby Bogaert 《PloS one》2012,7(10)
Background
The CRM197-conjugated 7-valent pneumococcal vaccine (PCV7) is protective against vaccine serotype disease and nasopharyngeal carriage. Data on PCV7-induced mucosal antibodies in relation to systemic or natural anticapsular antibodies are scarce.Methods
In a randomized controlled setting, children received PCV7 at age 2 and 4 months (2-dose group), at age 2, 4 and 11 months (2+1-dose group) or no PCV7 (control group). From 188 children paired saliva samples were collected at 12 and 24 months of age. From a subgroup of 15 immunized children also serum samples were collected. IgG and IgA antibody-levels were measured by multiplex immunoassay.Results
At 12 months, both vaccine groups showed higher serum and saliva IgG-levels against vaccine serotypes compared with controls which sustained until 24 months for most serotypes. Salivary IgG-levels were 10–20-fold lower compared to serum IgG, however, serum and saliva IgG-levels were highly correlated. Serum and salivary IgA-levels were higher in both vaccine groups at 12 months compared with controls, except for serotype 19F. Higher salivary IgA levels remained present for most serotypes in the 2+1-dose group until 24 months, but not in the 2-dose group. Salivary IgA more than IgG, increased after documented carriage of serotypes 6B, 19F and 23F In contrast to IgG, salivary IgA-levels were comparable with serum, suggesting local IgA-production.Conclusions
PCV7 vaccination results in significant increases in salivary IgG and IgA-levels, which are more pronounced for IgG when compared to controls. In contrast, salivary anticapsular IgA-levels seemed to respond more to natural boosting. Salivary IgG and IgA-levels correlate well with systemic antibodies, suggesting saliva might be useful as potential future surveillance tool. 相似文献14.
15.
16.
A. S. Kraus R. A. Spasoff E. J. Beattie D. E. Holden J. S. Lawson M. Rodenburg G. M. Woodcock 《CMAJ》1977,116(9):1007-1009
Little information is available on the health status of persons 85 years or over. Recent United States data indicate that 20% of individuals 85 years of age or over reside in nursing and personal care homes and that among these institutional residents 31% are bedfast, 11% are chairfast and 71% manifest evidence of senility. An investigation into the health of persons 85 years of age or over in two Ontario counties revealed that 39% resided in long-stay institutions, and that one of the main differences between old people living independently in the community and those in institutions was the higher proportion of the latter needing help with the activities of daily living and showing mental disorientation. Of those individuals 85 years of age or over in institutions for 1 year, 26% acknowledged often feeling depressed and 18% acknowledged often wishing they were dead, but these tendencies were not more frequent in those 85 years of age or over than in those 65 to 84 years of age. These findings have implications for service and research needs and attitudes towards death. 相似文献
17.
Charikleia Schoina Sander Y. A. Rodenburg Harold J. G. Meijer Michael F. Seidl Lysette T. Lacambra Klaas Bouwmeester Francine Govers 《Molecular Plant Pathology》2021,22(5):551-563
Pathogens deploy a wide range of pathogenicity factors, including a plethora of proteases, to modify host tissue or manipulate host defences. Metalloproteases (MPs) have been implicated in virulence in several animal and plant pathogens. Here we investigated the repertoire of MPs in 46 stramenopile species including 37 oomycetes, 5 diatoms, and 4 brown algae. Screening their complete proteomes using hidden Markov models (HMMs) trained for MP detection resulted in over 4,000 MPs, with most species having between 65 and 100 putative MPs. Classification in clans and families according to the MEROPS database showed a highly diverse MP repertoire in each species. Analyses of domain composition, orthologous groups, distribution, and abundance within the stramenopile lineage revealed a few oomycete-specific MPs and MPs potentially related to lifestyle. In-depth analyses of MPs in the plant pathogen Phytophthora infestans revealed 91 MPs, divided over 21 protein families, including 25 MPs with a predicted signal peptide or signal anchor. Expression profiling showed different patterns of MP gene expression during pre-infection and infection stages. When expressed in leaves of Nicotiana benthamiana, 12 MPs changed the sizes of lesions caused by inoculation with P. infestans; with 9 MPs the lesions were larger, suggesting a positive effect on the virulence of P. infestans, while 3 MPs had a negative effect, resulting in smaller lesions. To the best of our knowledge, this is the first systematic inventory of MPs in oomycetes and the first study pinpointing MPs as potential pathogenicity factors in Phytophthora. 相似文献
18.
Michael Wong‐Stringer Onkar S. Game Joel A. Smith Thomas J. Routledge Bakhet A. Alqurashy Benjamin G. Freestone Andrew J. Parnell Naoum Vaenas Vikas Kumar Majed O. A. Alawad Ahmed Iraqi Cornelia Rodenburg David G. Lidzey 《Liver Transplantation》2018,8(24)
An encapsulation system comprising of a UV‐curable epoxy, a solution processed polymer interlayer, and a glass cover‐slip, is used to increase the stability of methylammonium lead triiodide (CH3NH3PbI3) perovskite planar inverted architecture photovoltaic (PV) devices. It is found this encapsulation system acts as an efficient barrier to extrinsic degradation processes (ingress of moisture and oxygen), and that the polymer acts as a barrier that protects the PV device from the epoxy before it is fully cured. This results in devices that maintain 80% of their initial power conversion efficiency after 1000 h of AM1.5 irradiation. Such devices are used as a benchmark and are compared with devices having initially enhanced efficiency as a result of a solvent annealing process. It is found that such solvent‐annealed devices undergo enhanced burn‐in and have a reduced long‐term efficiency, a result demonstrating that initially enhanced device efficiency does not necessarily result in long‐term stability. 相似文献
19.
Thatjana Gardeitchik Miski Mohamed Benedetta Ruzzenente Daniela Karall Sergio Guerrero-Castillo Daisy Dalloyaux Mariël van den Brand Sanne van Kraaij Ellyze van Asbeck Zahra Assouline Marlene Rio Pascale de Lonlay Sabine Scholl-Buergi David F.G.J. Wolthuis Alexander Hoischen Richard J. Rodenburg Wolfgang Sperl Zsolt Urban Eva Morava 《American journal of human genetics》2018,102(4):685-695
20.
On-farm assessment of laying hen welfare: a comparison of one environment-based and two animal-based methods 总被引:1,自引:0,他引:1
H. Mollenhorst T.B. Rodenburg E.A.M. Bokkers P. Koene I.J.M. de Boer 《Applied animal behaviour science》2005,90(3-4):277-291
Methods available to assess animal welfare at farm level are based on a range of welfare parameters, which can be divided into two categories, environment-based and animal-based parameters. The first category describes features of the environment and management, which can be considered prerequisites for welfare. The second category records animals’ responses to that particular environment and management more directly. Objective of this study was to validate a mainly environment-based method, the animal needs index (ANI), with animal-based methods: behavioural observations and feather condition scores (FCS). The study was conducted on 20 commercial laying hen farms, 10 farms with battery cages and 10 farms with deep litter systems. During a 1-day visit on each farm, ANI was assessed, FCS was scored, and behavioural observations were performed. Instantaneous scan sampling and continuous focal sampling were used to assess the time spent on different behaviours and the occurrence of event behaviours. Data from behavioural observations and FCS were reduced with principal factor analysis. This resulted in two factors for each method. Significant positive correlations were found between ANI, on the one hand, and ‘movement’ and ‘comfort’, two factors from behavioural observations, on the other hand. A significant negative correlation was found between ANI and ‘wing damage’ (from FCS). The results of this study show that ANI is valid and sensitive enough to show differences in animal welfare between housing systems, whereas differences in welfare within housing systems cannot be shown. In conclusion, ANI is an appropriate method for assessment of laying hen welfare on a large number of farms with different housing systems. 相似文献