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11.
Rooryck C Diaz-Font A Osborn DP Chabchoub E Hernandez-Hernandez V Shamseldin H Kenny J Waters A Jenkins D Kaissi AA Leal GF Dallapiccola B Carnevale F Bitner-Glindzicz M Lees M Hennekam R Stanier P Burns AJ Peeters H Alkuraya FS Beales PL 《Nature genetics》2011,43(3):197-203
3MC syndrome has been proposed as a unifying term encompassing the overlapping Carnevale, Mingarelli, Malpuech and Michels syndromes. These rare autosomal recessive disorders exhibit a spectrum of developmental features, including characteristic facial dysmorphism, cleft lip and/or palate, craniosynostosis, learning disability and genital, limb and vesicorenal anomalies. Here we studied 11 families with 3MC syndrome and identified two mutated genes, COLEC11 and MASP1, both of which encode proteins in the lectin complement pathway (collectin kidney 1 (CL-K1) and MASP-1 and MASP-3, respectively). CL-K1 is highly expressed in embryonic murine craniofacial cartilage, heart, bronchi, kidney and vertebral bodies. Zebrafish morphants for either gene develop pigmentary defects and severe craniofacial abnormalities. Finally, we show that CL-K1 serves as a guidance cue for neural crest cell migration. Together, these findings demonstrate a role for complement pathway factors in fundamental developmental processes and in the etiology of 3MC syndrome. 相似文献
12.
用最小二乘拟合法,将稀土区的能谱实验数据,拟合不同的能谱公式,从而证明E=a〔(1+bI(I+1))~(1/2)-1〕是符合得较好的,本文还推导了与上述能谱相应的两类转动惯量,并以此与实验计算值进行比较,结果仍很满意. 相似文献
13.
对 2 0 0 MW核供热堆装载模式 (燃料组件布置、可燃毒物棒根数和可燃毒物质量分数配置 )进行了优化。利用模拟退火算法和先进格林函数节块法进行多步燃耗优化计算。引入敏感性系数 ,并通过敏感性分析的方法决定优化参数 ,因此在单目标和多目标优化时均取得了明显的效果。对组件布置和可燃毒物质量分数的优化计算结果表明 ,在不改变原有的富集度和组件类型的前提下 ,与参考值相比 ,优化后的循环长度、功率峰因子和卸料燃耗均有明显的改善。该燃料管理方法不仅可用于低温堆而且也可以推广到压水堆 相似文献
14.
A population of ventral neural tube cells has recently been shown to migrate out of the hind brain neural tube via the vagus
nerve and contribute to the developing gastrointestinal tract. Since liver is also innervated by the vagus nerve, we sought
to determine if these cells also migrate into the liver. Ventral neural tube cells in the caudal hindbrain of chick embryos
were tagged with a replication-deficient retroviral vector containing the LacZ gene on embryonic day 2. Embryos were processed
for detection of labeled cells on embryonic day 5 and 11. Labeled cells were seen in the liver on both days and identified
as hepatocytes. Previously, it was believed that all hepatocytes develop from the gut endoderm. Results of the present study
show an additional source for the formation of liver cells.
Received 25 August 1998; received after revision 5 November 1998; accepted 5 November 1998 相似文献
15.
N. M. Ali 《Cellular and molecular life sciences : CMLS》1982,38(2):239-241
Summary Histochemical determination of neuroglia investing parasites in naturally infected fish depends upon recognition of the highly soluble or diffusible nature of the cytoplasm.Acknowledgment. M.R.L. Johnston advised me during the course of this work. 相似文献
16.
Cells and cell-free solutions of the culture filtrate of the bacterial symbiont, Xenorhabdus nematophila taken from the entomopathogenic nematode Steinernema carpocapsae in aqueous broth suspensions were lethal to larvae of the diamondback moth Plutella xylostella. Their application on leaves of Chinese cabbage indicated that the cells can penetrate into the insects in the absence of the nematode vector. Cell-free solutions containing metabolites were also proved as effective as bacterial cells suspension. The application of aqueous suspensions of cells of X.nematophila or solutions containing its toxic metabolites to the leaves represents a possible new strategy for controlling insect pests on foliage. 相似文献
17.
18.
Jajcay‘s studies( 1993 ; 1994) on the automorphism groups of Cayley maps yielded a new product of groups, which he called, rotary product. Using this product, we define a hyperoperation ⊙ on the group Syme (G) , the stabilizer of the identity e ∈ G in the group Sym (G) . We prove that ( Syme (G) , ⊙) is a hypergroup and characterize the subhypergroups of this hypergroup.Finally, we show that the set of all subhypergroups of Syme ( G ) constitute a lattice under ordinary join and meet and that the minimal elements of order two of this lattice is a subgroup of Aut (G) . 相似文献
19.
Qiu Baoli Susan A. Coats Ren Shunxiang Ali M. Idris Xu Caixia Judith K. Brown 《自然科学进展》2007,17(6):645-654
Phylogenetic relationships for Bemisia tabaci were reconstructed by analysis of a ~780 bp fragment of the mitochondrial cytochrome oxidase I (mtCOI) gene with an emphasis on geographic range and distribution among eight eudicot plant families that are common hosts of B. tabaci worldwide to elucidate key phylogeographic linkages between populations extant in China (n=31) and India (n=34). Bootstrap values for the Maximum Parsimony tree were highly robust for all major nodes involving the major Asian clade, subgroups, and sister groups within, at 92%—100%. Between-clade distances for the Southeast Asia and three other major clades, e.g. from sub-Sahara Africa, North Africa-Mediterranean, and the Americas, were approximately >16% divergent. Two major Asian subgroups (I, II) were resolved, which represented populations indigenous to the region, comprising two (I a, I b) and five (II a—e) sister groups, respectively, which diverged by 11%. Two distinct populations from sunflower in Hyderabad grouped separately within the two Asian subgroups. All other populations grouped uniquely within Asian subgroup II or I. The “B” biotype was identified in 23 collections from China at 97.3%—99.5% nucleotide identity with “B” biotype reference sequences; it was not identified in collections from India. The majority of haplotypes were associated with 3—4 plant families, with one exception that for sister group IId (sesame, India), it might be monophagous. Thus, B. tabaci from the southeastern and near eastern regions of the Asian continent comprise of a large number of ancestral, richly divergent, mostly polyphagous populations. This region is therefore hypothesized to constitute an important Old World center of diversification for the B. tabaci complex, together with sub-Saharan Africa. 相似文献
20.
Thiamine derivatives bind messenger RNAs directly to regulate bacterial gene expression 总被引:51,自引:0,他引:51
Although proteins fulfil most of the requirements that biology has for structural and functional components such as enzymes and receptors, RNA can also serve in these capacities. For example, RNA has sufficient structural plasticity to form ribozyme and receptor elements that exhibit considerable enzymatic power and binding specificity. Moreover, these activities can be combined to create allosteric ribozymes that are modulated by effector molecules. It has also been proposed that certain messenger RNAs might use allosteric mechanisms to mediate regulatory responses depending on specific metabolites. We report here that mRNAs encoding enzymes involved in thiamine (vitamin B(1)) biosynthesis in Escherichia coli can bind thiamine or its pyrophosphate derivative without the need for protein cofactors. The mRNA-effector complex adopts a distinct structure that sequesters the ribosome-binding site and leads to a reduction in gene expression. This metabolite-sensing regulatory system provides an example of a 'riboswitch' whose evolutionary origin might pre-date the emergence of proteins. 相似文献