The gene encoding ganglioside-induced differentiation-associated protein 1 is mutated in axonal Charcot-Marie-Tooth type 4A disease.

We identified three distinct mutations and six mutant alleles in GDAP1 in three families with axonal Charcot-Marie-Tooth (CMT) neuropathy and vocal cord paresis, which were previously linked to the CMT4A locus on chromosome 8q21.1. These results establish the molecular etiology of CMT4A (MIM 214400) and suggest that it may be associated with both axonal and demyelinating phenotypes.     

神经网络在橡胶硫化温度预测中的应用

本文针对难以通过在线测温实现等效硫化控制的情况,在确定硫化条件的直接测温实验基础上,提出了用神经网络模型预测实际生产过程中硫化制品内部温度的方法．在该方法中将所测温度值作为样本提供给神经网络学习,温度采集时刻及易测外部温度作为网络输入,输出值则是在硫化时制品难测温点处不同时刻的内温．神经网络通过学习取得了良好的效果,网络输出的温度值将成为等效硫化计算和硫化质量智能控制的有价值的依据．     

A postsynaptic α, 2-receptor: The alpha-adrenergic receptor of hamster white fat cells

Summary The effects of selected ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">-agonists and ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">-antagonists on theophylline-induced lipolysis were investigate in isolated hamster white fat cells ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">₂-Agonists (tramazoline, clonidine) inhibited theophylline-induced lipolysis while an ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">₂-agonist (methoxamine) was without any effect. The inhibitory effect of ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">₂-agonists was suppressed by yohimbine (ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">₂-antagonist), whereas ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">₂-antagonists were inefficient. This result implies that the ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">-adrenergic receptor of hamster fat cells is of the ntent/v411047836576242/xxlarge945.gif" alt="agr" align="BASELINE" BORDER="0">₂-type, although located postsynaptically.Acknowledgments. This work was supported by grants from CNRS (ERA 412) and DGRST (grant No. 787 1078). We thank M. Dauzats for excellent technical assistance. We thank Prof. H. Schmitt for tramazoline and AR-C 239 and for helpful discussion.     

Dissociation of glucocorticoid effects of C-21 steroids at high concentrations in thymocytes

Summary A dissociation between inhibition of RNA synthesis and cell lysis was observed when thymocytes of adrenalectomized rats were incubated with high concentrations of pregn-4-ene-11ntent/j5m6t5n61032j21n/xxlarge946.gif" alt="beta" align="MIDDLE" BORDER="0">-ol-3, 20-dione and pregna-1,4-diene-11ntent/j5m6t5n61032j21n/xxlarge946.gif" alt="beta" align="MIDDLE" BORDER="0">-ol-3,20-dione. In contrast, no dissociation of these effects was found with the typical glucocorticoids cortisol and corticosterone, nor with their 1,4-diene analogs under the same conditions.Acknowledgments. This work was supported by grants from the Consejo Nacional de Investigaciones Científicas y Técnicas de la República Argentina to the Instituto de Biologia y Medicina Experimental and the Programa de Regulación Hormonal y Metabólica as well as by financial help from the Secretaría de Ciencia y Tecnología.     

Critical role of extracellular vesicles in modulating the cellular effects of cytokines

Under physiological and pathological conditions, extracellular vesicles (EVs) are present in the extracellular compartment simultaneously with soluble mediators. We hypothesized that cytokine effects may be modulated by EVs, the recently recognized conveyors of intercellular messages. In order to test this hypothesis, human monocyte cells were incubated with CCRF acute lymphoblastic leukemia cell line-derived EVs with or without the addition of recombinant human TNF, and global gene expression changes were analyzed. EVs alone regulated the expression of numerous genes related to inflammation and signaling. In combination, the effects of EVs and TNF were additive, antagonistic, or independent. The differential effects of EVs and TNF or their simultaneous presence were also validated by Taqman assays and ntent-type=ReferenceWorkEntry&sortOrder=relevance' class='reference-link webtrekk-track' gaCategory="internal-link" gaLabel="ELISA" gaAction="reference keyword">ELISA, and by testing different populations of purified EVs. In the case of the paramount chemokine IL-8, we were able to demonstrate a synergistic upregulation by purified EVs and TNF. Our data suggest that neglecting the modulating role of EVs on the effects of soluble mediators may skew experimental results. On the other hand, considering the combined effects of cytokines and EVs may prove therapeutically useful by targeting both compartments at the same time.     

Cell-free expression and in meso crystallisation of an integral membrane kinase for structure determination

Membrane proteins are key elements in cell physiology and drug targeting, but getting a high-resolution structure by crystallographic means is still enormously challenging. Novel strategies are in big demand to facilitate the structure determination process that will ultimately hasten the day when sequence information alone can provide a three-dimensional model. Cell-free or in vitro expression enables rapid access to large quantities of high-quality membrane proteins suitable for an array of applications. Despite its impressive efficiency, to date only two membrane proteins produced by the in vitro approach have yielded crystal structures. Here, we have analysed synergies of cell-free expression and crystallisation in lipid mesophases for generating an X-ray structure of the integral membrane enzyme diacylglycerol kinase to 2.28-Å resolution. The quality of cellular and cell-free-expressed kinase samples has been evaluated systematically by comparing (1) spectroscopic properties, (2) purity and oligomer formation, (3) lipid content and (4) functionality. DgkA is the first membrane enzyme crystallised based on cell-free expression. The study provides a basic standard for the crystallisation of cell-free-expressed membrane proteins and the methods detailed here should prove generally useful and contribute to accelerating the pace at which membrane protein structures are solved.     

A new parasitoid (Hymenoptera: Braconidae: Aphidiinae) of the invasive bamboo aphids Takecallis spp. (Hemiptera: Aphididae) from Western Europe

A long-term survey of tritrophic (plant–aphid–parasitoid) associations in the urban ecosystems of Lleida (Catalonia) and Paris (France) resulted in the detection of associations of two bamboo aphids, Takecallis arundinariae (Essig) and Takecallis taiwanus (Takahashi), respectively, with a new aphid parasitoid species. Trioxys remaudierei Starý & Rakhshani sp. nov. is described and illustrated as a unique parasitoid of Takecallis aphids outside the area of their origin. The new species is easily distinguishable from its congeners in having the ventral prongs of the abdomen fused over two-thirds of their length, then bifurcated towards the tip. The only morphologically similar species is Trioxys betulae (Marshall), which exhibits a clearly different prong shape (and has a different host range, Symydobius Mordvilko and Clethrobius Mordvilko). The new species is compared with allied taxa associated with bamboo aphids. The occurrence of Takecallis taiwanus on bamboo is recorded in France for the first time.

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Babylonian solar theory on the Antikythera mechanism

This article analyzes the angular spacing of the degree marks on the zodiac scale of the Antikythera mechanism and demonstrates that over the entire preserved 88° of the zodiac, the marks are systematically placed too close together to be consistent with a uniform distribution over 360°. Thus, in some other part of the zodiac scale (not preserved), the degree marks have been spaced farther apart. By contrast, the day marks on the Egyptian calendar scale are spaced uniformly, apart from minor errors. A solar equation of center is apparent which rises by nearly 2.7° over the preserved portion of the zodiac. The placement of the degree marks indicates that, in the preserved portion of the zodiac, the Sun was considered to run at a uniform pace of about 30° per synodic month, which is consistent with the Sun’s speed in the fast zone of the Babylonian solar theory of System A.