Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm

Loeys-Dietz syndrome (LDS) associates with a tissue signature for high transforming growth factor (TGF)-β signaling but is often caused by heterozygous mutations in genes encoding positive effectors of TGF-β signaling, including either subunit of the TGF-β receptor or SMAD3, thereby engendering controversy regarding the mechanism of disease. Here, we report heterozygous mutations or deletions in the gene encoding the TGF-β2 ligand for a phenotype within the LDS spectrum and show upregulation of TGF-β signaling in aortic tissue from affected individuals. Furthermore, haploinsufficient Tgfb2(+/-) mice have aortic root aneurysm and biochemical evidence of increased canonical and noncanonical TGF-β signaling. Mice that harbor both a mutant Marfan syndrome (MFS) allele (Fbn1(C1039G/+)) and Tgfb2 haploinsufficiency show increased TGF-β signaling and phenotypic worsening in association with normalization of TGF-β2 expression and high expression of TGF-β1. Taken together, these data support the hypothesis that compensatory autocrine and/or paracrine events contribute to the pathogenesis of TGF-β-mediated vasculopathies.     

A20 (TNFAIP3) deficiency in myeloid cells triggers erosive polyarthritis resembling rheumatoid arthritis

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.     

Quantum Mechanics and Computation

In quantum computation non classical features such as superposition states and entanglement are used to solve problems in new ways, impossible on classical digital computers.We illustrate by Deutsch algorithm how a quantum computer can use superposition states to outperform any classical computer. We comment on the view of a quantum computer as a massive parallel computer and recall Amdahls law for a classical parallel computer. We argue that the view on quantum computation as a massive parallel computation disregards the presence of entanglement in a general quantum computation and the non classical way in which parallel results are combined to obtain the final output.     

Mathematical Arguments in Context

Except in very poor mathematical contexts, mathematical arguments do not stand in isolation of other mathematical arguments. Rather, they form trains of formal and informal arguments, adding up to interconnected theorems, theories and eventually entire fields. This paper critically comments on some common views on the relation between formal and informal mathematical arguments, most particularly applications of Toulmin’s argumentation model, and launches a number of alternative ideas of presentation inviting the contextualization of pieces of mathematical reasoning within encompassing bodies of explicit and implicit, formal and informal background knowledge.     

催化氧化净化黄磷尾气中的磷和硫

利用黄磷尾气生产甲酸、乙酸以及甲醇等高附加值产品，净化预处理成为制药其应用的瓶颈问题。研究了用普通活性炭和自制催化剂KU2催化氧化净化黄磷尾气的方法；讨论了固定床系统中常压下、温度20~140℃，催化剂上磷和硫化氢的吸附特征。KU2和活性炭都能通过催化氧化过程有效脱除黄磷尾气中的P4，PH3和H2S杂质，随着反应温度和氧含量增加可显著提高净化效果，在最优条件下，经催化氧化净化后的总磷含量＜5mg/m3，硫化氢＜5mg/m3，失效后的催化剂易于再生，催化剂经多次再生后催化性能恢复良好；提出了活性炭固定床稳定     

Aspects of Romanian Early-Middle Jurassic palaeobotany and palynology. Part VII. Successions and floras

The Jurassic floras of Romania occur in the Carpathian Mountains and in Dobrogea (Eastern Romania). In the South Carpathians, diverse and well preserved floras have been recorded since the middle of the 19th Century in the Resita, Sirinia, Presacina, CernaJiu, Holbav and Cristian Basins. The ages of these floras range between Hettangian and Sinemurian, with representatives belonging to Bryophytes, Pteridophytes and Gymnosperms. Excellent preservation and diversity were recorded in Anina, in the central area of the Resita Basin, a possible fossile-Lagerst?tte locality. The 3D collecting opportunities due to underground coal mining permitted detailed phytostratigraphical studies. These studies showed a floral change at the Hettangian-Sinemurian boundary, related to climate change. The Jurassic coal basins of the South Carpathians show both intramontainous and paralic features. The Resita Basin is a typical intramountainous depression basin, while the other basins are influenced by the marine realm. From a paleogeographical point of view, the Romanian Early and Middle Jurassic floras occurred on the northern frame of the Tethys realm, floristic features indicating the Eurosinian Province. One of the closest floras in terms of floristic similarities is the Iranian Early Jurassic flora. In the Apuseni Mountains, the HettangianSinemurian flora is less well-preserved and diverse than those of the South Carpathians. The Middle Jurassic flora of Romania is confined to Dobrogea (Eastern Romania), where a bennettite-dominated assemblage with low diversity and preservation was recorded.