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排序方式: 共有187条查询结果,搜索用时 153 毫秒
1.
Aloysius Domingo David Amar Karen Grütz Lillian V. Lee Raymond Rosales Norbert Brüggemann Roland Dominic Jamora Eva Cutiongco-dela Paz Arndt Rolfs Dirk Dressler Uwe Walter Dimitri Krainc Katja Lohmann Ron Shamir Christine Klein Ana Westenberger 《Cellular and molecular life sciences : CMLS》2016,73(16):3205-3215
2.
Archive for History of Exact Sciences - Earlier than the Arabic-Latin transfer of Ptolemaic astronomy via the Iberian peninsula, a serious occupation with Arabic astronomy by Latin scholars took... 相似文献
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Roscioli T Kamsteeg EJ Buysse K Maystadt I van Reeuwijk J van den Elzen C van Beusekom E Riemersma M Pfundt R Vissers LE Schraders M Altunoglu U Buckley MF Brunner HG Grisart B Zhou H Veltman JA Gilissen C Mancini GM Delrée P Willemsen MA Ramadža DP Chitayat D Bennett C Sheridan E Peeters EA Tan-Sindhunata GM de Die-Smulders CE Devriendt K Kayserili H El-Hashash OA Stemple DL Lefeber DJ Lin YY van Bokhoven H 《Nature genetics》2012,44(5):581-585
Walker-Warburg syndrome (WWS) is an autosomal recessive multisystem disorder characterized by complex eye and brain abnormalities with congenital muscular dystrophy (CMD) and aberrant a-dystroglycan glycosylation. Here we report mutations in the ISPD gene (encoding isoprenoid synthase domain containing) as the second most common cause of WWS. Bacterial IspD is a nucleotidyl transferase belonging to a large glycosyltransferase family, but the role of the orthologous protein in chordates is obscure to date, as this phylum does not have the corresponding non-mevalonate isoprenoid biosynthesis pathway. Knockdown of ispd in zebrafish recapitulates the human WWS phenotype with hydrocephalus, reduced eye size, muscle degeneration and hypoglycosylated a-dystroglycan. These results implicate ISPD in a-dystroglycan glycosylation in maintaining sarcolemma integrity in vertebrates. 相似文献
5.
Dirk M. Walther Doron Rapaport Jan Tommassen 《Cellular and molecular life sciences : CMLS》2009,66(17):2789-2804
Membrane-embedded β-barrel proteins span the membrane via multiple amphipathic β-strands arranged in a cylindrical shape.
These proteins are found in the outer membranes of Gram-negative bacteria, mitochondria and chloroplasts. This situation is
thought to reflect the evolutionary origin of mitochondria and chloroplasts from Gram-negative bacterial endosymbionts. β-barrel
proteins fulfil a variety of functions; among them are pore-forming proteins that allow the flux of metabolites across the
membrane by passive diffusion, active transporters of siderophores, enzymes, structural proteins, and proteins that mediate
protein translocation across or insertion into membranes. The biogenesis process of these proteins combines evolutionary conservation
of the central elements with some noticeable differences in signals and machineries. This review summarizes our current knowledge
of the functions and biogenesis of this special family of proteins. 相似文献
6.
Matmati M Jacques P Maelfait J Verheugen E Kool M Sze M Geboes L Louagie E Mc Guire C Vereecke L Chu Y Boon L Staelens S Matthys P Lambrecht BN Schmidt-Supprian M Pasparakis M Elewaut D Beyaert R van Loo G 《Nature genetics》2011,43(9):908-912
A20 (TNFAIP3) is a protein that is involved in the negative feedback regulation of NF-κB signaling in response to specific proinflammatory stimuli in different cell types and has been suggested as a susceptibility gene for rheumatoid arthritis. To define the contribution of A20 to rheumatoid arthritis pathology, we generated myeloid-specific A20-deficient mice and show that specific ablation of Tnfaip3 in myeloid cells results in spontaneous development of a severe destructive polyarthritis with many features of rheumatoid arthritis. Myeloid-A20-deficient mice have high levels of inflammatory cytokines in their serum, consistent with a sustained NF-κB activation and higher TNF production by macrophages. Destructive polyarthritis in myeloid A20 knockout mice was TLR4-MyD88 and IL-6 dependent but was TNF independent. Myeloid A20 deficiency also promoted osteoclastogenesis in mice. Together, these observations indicate a critical and cell-specific function for A20 in the etiology of rheumatoid arthritis, supporting the idea of developing A20 modulatory drugs as cell-targeted therapies. 相似文献
7.
C. Schubert 《Cellular and molecular life sciences : CMLS》2009,66(7):1178-1197
The Williams-Beuren syndrome is a genomic disorder (prevalence: 1/7,500 to 1/20,000), caused by a hemizygous contiguous gene
deletion on chromosome 7q11.23. Typical symptoms comprise supravalvular aortic stenosis, mental retardation, overfriendliness
and visuospatial impairment. The common deletion sizes range of 1.5–1.8 mega base pairs (Mb), encompassing app. 28 genes.
For a few genes, a genotype-phenotype correlation has been established. The best-explored gene within this region is the elastin
gene; its haploinsufficiency causes arterial stenosis. The region of the Williams-Beuren syndrome consists of a single copy
gene region (~1.2 Mb) flanked by repetitive sequences – Low Copy Repeats (LCR). The deletions arise as a consequence of misalignment
of these repetitive sequences during meiosis and a following unequal crossing over due to high similarity of LCRs. This review
presents an overview of the Williams-Beuren syndrome region considering the genomic assembly, chromosomal rearrangements and
their mechanisms (i.e. deletions, duplications, inversions) and evolutionary and historical aspects.
Received 11 July 2008; received after revision 15 October 2008; accepted 16 October 2008 相似文献
8.
本文着重研究了自还原性胺钨盐的性质及其氢还原,发现,将自还原与一般氢还原适当结合,可以高效率地制备比表面积大于20m~2/g的极细、超细钨粉。对自还原的本性及该类钨盐在制取极细、超细钨粉方面的应用前景进行了讨论。 相似文献
9.
Non-uniform rational B-spline (NURBS) curves and surfaces are very important tools for modelling curves and surfaces. Several important details, such as the choice of the sample points, of the parameterization, and of the termination condition, are however not well described. These details have a great influence on the performance of the approximation algorithm, both in terms of quality as well as time and space usage. This paper described how to sample points, examining two standard parameterizations: equidistant and chordal. A new and local parameterization, namely an adaptive equidistant model, was proposed, which enhances the equidistant model, Localization can also be used to enhance the chordal parameterizatfon, For NURBS surfaces, one must choose which direction will be approximated first and must pay special attention to surfaces of degree 1 which have to be handled as a special case. 相似文献
10.
Towards sustainability in world fisheries 总被引:70,自引:0,他引:70
Pauly D Christensen V Guénette S Pitcher TJ Sumaila UR Walters CJ Watson R Zeller D 《Nature》2002,418(6898):689-695
Fisheries have rarely been 'sustainable'. Rather, fishing has induced serial depletions, long masked by improved technology, geographic expansion and exploitation of previously spurned species lower in the food web. With global catches declining since the late 1980s, continuation of present trends will lead to supply shortfall, for which aquaculture cannot be expected to compensate, and may well exacerbate. Reducing fishing capacity to appropriate levels will require strong reductions of subsidies. Zoning the oceans into unfished marine reserves and areas with limited levels of fishing effort would allow sustainable fisheries, based on resources embedded in functional, diverse ecosystems. 相似文献