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There is no treatment for fibrotic disease is a significant cause of mortality. CCN2 Members of the CCN family of matricellular proteins have a characteristic four domain structure. CCN2 (connective tissue growth factor) is believed to play an essential role in fibrogenesis. In a recent paper, data are provided that CCN5 (wisp2), which lacks the carboxy-terminal heparin-binding domain shared by the other CCN proteins, may act as a dominant-negative protein to suppress CCN2-mediated fibrogenesis. These data are consistent with the notion that different CCN proteins may enhance or suppress each other’s action and also suggest that CCN5, may be used as a novel anti-fibrotic therapy.  相似文献   

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IntroductionSignificant pulmonary vascular disease is a leading cause of death in patients with scleroderma, and early detection and early medical intervention are important, as they may delay disease progression and improve survival and quality of life. Although several biomarkers have been proposed, there remains a need to define a reliable biomarker of early pulmonary vascular disease and subsequent development of pulmonary hypertension (PH). The purpose of this study was to define potential biomarkers for clinically significant pulmonary vascular disease in patients with scleroderma.MethodsThe circulating growth factors basic fibroblast growth factor, placental growth factor (PlGF), vascular endothelial growth factor (VEGF), hepatocyte growth factor, and soluble VEGF receptor 1 (sFlt-1), as well as cytokines (interleukin [IL]-1β IL-2, IL-4, IL-5, IL-8, IL-10, IL-12, IL-13, tumor necrosis factor-α, and interferon-γ), were quantified in patients with scleroderma with PH (n = 37) or without PH (n = 40). In non-parametric unadjusted analyses, we examined associations of growth factor and cytokine levels with PH. In a subset of each group, a second set of earlier samples, drawn 3.0±1.6 years earlier, were assessed to determine the changes over time.ResultssFlt-1 (p = 0.02) and PlGF (p = 0.02) were higher in the PH than in the non-PH group. sFlt-1 (ρ = 0.3245; p = 0.01) positively correlated with right ventricular systolic pressure. Both PlGF (p = 0.03) and sFlt-1 (p = 0.04) positively correlated with the ratio of forced vital capacity to diffusing capacity for carbon monoxide (DLCO), and both inversely correlated with DLCO (p = 0.01). Both PlGF and sFlt-1 levels were stable over time in the control population.ConclusionsOur study demonstrated clear associations between regulators of angiogenesis (sFlt-1 and PlGF) and measures of PH in scleroderma and that these growth factors are potential biomarkers for PH in patients with scleroderma. Larger longitudinal studies are required for validation of our results.

Electronic supplementary material

The online version of this article (doi:10.1186/s13075-015-0712-4) contains supplementary material, which is available to authorized users.  相似文献   

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Alzheimer Disease (AD) is the most common neurodegenerative disorder worldwide, and account for 60% to 70% of all cases of progressive cognitive impairment in elderly patients. At the microscopic level distinctive features of AD are neurons and synapses degeneration, together with extensive amounts of senile plaques and neurofibrillars tangles. The degenerative process probably starts 20–30 years before the clinical onset of the disease. Senile plaques are composed of a central core of amyloid β peptide, Aβ, derived from the metabolism of the larger amyloid precursor protein, APP, which is expressed not only in the brain, but even in non neuronal tissues. More than 30 years ago, some studies reported that human platelets express APP and all the enzymatic activities necessary to process this protein through the same pathways described in the brain. Since then a large number of evidence has been accumulated to suggest that platelets may be a good peripheral model to study the metabolism of APP, and the pathophysiology of the onset of AD. In this review, we will summarize the current knowledge on the involvement of platelets in Alzheimer Disease. Although platelets are generally accepted as a suitable model for AD, the current scientific interest on this model is very high, because many concepts still remain debated and controversial. At the same time, however, these still unsolved divergences mirror a difficulty to establish constant parameters to better defined the role of platelets in AD.  相似文献   

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Conservation that’s more than skin-deep: alligator farming   总被引:1,自引:0,他引:1  
Wildlife farming is a contentious conservation measure. In Louisiana alligator farming has generated significant conservation gains. This case study is used to test several assumptions employed in debates about wildlife farming. These include whether farming ‘floods’ the market to depress prices and deter poaching, whether it encourages wild harvest and whether it can compete against wild harvest. Data from over three decades is used to model harvest behaviour with OLS and SUR models. This shows strong separation between the market between farmed and wild alligator skins. Immense rises in farmed output have not caused prices to collapse, however poaching has collapsed. This highlights that farming can have important non-price effects on poaching. Assumptions that are commonly used to debate wildlife farming are not supported in this example. Such assumptions, including open-access of the wildlife, inert and exogenous wildlife managers and excluding indirect benefits of wildlife farming tend to bias policy away from farming. Using these assumptions makes it harder to identify cases where wildlife farming could assist conservation objectives.  相似文献   

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The lack of species inventory data for most marine habitats currently hampers the objective management of marine biodiversity. There is thus a clear need to find reliable indicator taxa that can be targeted in marine conservation studies, providing cost-effective data for planning and monitoring. Using the rocky shores of the Solitary Islands Marine Park, NSW, Australia, as a model, I evaluated macroinvertebrates and determined which taxa (i) best reflected ecological patterns of the broader intertidal community; and (ii) were able to accurately predict species richness of assemblages at the headland scale. Both molluscs and crustaceans showed high levels of correlation with overall species richness. However, molluscs, and in particular prosobranchs, most closely reflected patterns in the community data and provided the most accurate predictions of species richness at the scale of the headland. The potential time savings of using molluscs in rapid assessments are considerable and relate to reductions in field time (by up to 40%) as well as the reduced need to invest time developing extensive taxonomic knowledge of other invertebrate groups. Molluscs are widespread and easily sampled, with stable taxonomy and well-known ecology relative to other marine invertebrate taxa. Their use as surrogates of biodiversity shows great potential for future marine conservation studies.  相似文献   

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Can ideology, as a widespread “expectation creator,” impact economic decisions? In two studies we investigated the influence of the Benevolent Sexism (BS) ideology (which dictates that men should provide for passive and nurtured women) on women’s economic decision-making. In Study 1, using a Dictator Game in which women decided how to share amounts of money with men, results of a Generalized Linear Mixed Model analysis show that higher endorsement of BS and contextual expectations of benevolence were associated with more very unequal offers. Similarly, in an Ultimatum Game in which women received monetary offers from men, Study 2’s Generalized Linear Mixed Model’s results revealed that BS led women to reject more very unequal offers. If women’s endorsement of BS ideology and expectations of benevolence prove contrary to reality, they may strike back at men. These findings show that BS ideology creates expectations that shape male-female relationships in a way that could be prejudicial to men.  相似文献   

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Mutations in PARK2 are considered a common cause of Parkinson’s disease (PD). To assess the frequency of PARK2 mutations in the Korean population, we screened the PARK2 gene in 83 Korean PD patients: two young onset (YO, ≤ 49), 32 middle onset (MO, 50–69) and 49 late onset (LO, ≥ 70). Detection of the point mutations was performed by direct sequencing of the PARK2 exons, and exonic rearrangements were analyzed using multiplex ligation-dependent probe amplification. Five known PARK2 variants were identified in 53 (63.9 %) of the Korean PD patients: two missense mutations (Y267H and M458L) and three polymorphisms (S167N, L272I and V380L). We also found an increased frequency of PARK2 variants in PD patients and a lowered PD age at onset (AAO) in those having two variants, suggesting that the genetic variation in PARK2 gene might be a genetic risk factor of PD in Korean population.  相似文献   

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Kerwyn Casey (“KC”) Huang is an Associate Professor at Stanford University, studying the physical nature of biological systems and the underpinnings of fundamental processes such as cell shape determination, cell division, and intracellular and microbial community organization. In this interview, KC discusses how the ability to pursue insights at scales from molecules to cellular communities can shed new light on longstanding questions, the necessity for new tools in exploring the microbiome, how to create an empowering lab environment, and why integrating chemistry with physics and biology can bring us closer to asking the right questions.  相似文献   

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Tuckerella japonica Ehara appears strongly associated with tea (Camellia sinensis (L.) Kuntze, Theaceae) and, due to certain cultural practices in tea production, has in fact become a world traveller, accompanying the greatly coveted tea plant as it spread across the planet. The history of tea production and culture, and its arrival in the USA, provides the backdrop for this traveller’s tale. Tuckerella japonica is morphologically similar to T. flabellifera Miller, described from Tasmania in Australia from Bedfordia salicina (Labill.) D.G. (Asteraceae). These two species have historically been misidentified as each other, creating inaccuracies in the collection records. The implications of this in terms of host plant lists and world distribution are discussed further, along with their morphological separation. The male and immature stages of T. japonica are described for the first time. Tuckerella xinglongensis Lin and Fu, from tea in China, is considered a junior synonym of T. japonica. The loss of the ancestral prostigmatan condition of three nymphal stages during ontogeny is confirmed for males of T. flabellifera, which do not retain a tritonymphal stage.  相似文献   

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Thomsen??s and Becker??s diseases are the most prevalent nondystrophic myotonias. Their frequency varies, according to different sources, from 1: 100000 to 1: 10000. Thomsen??s myotonia is autosomal dominant, and Becker??s myotonia is autosomal recessive. Both diseases result from mutations of the CLCN1 gene encoding chloride ion channels of skeletal muscles. Molecular genetic analysis of the CLCN1 gene has been performed in patients with diagnoses of nondystrophic Thomsen??s and Becker??s myotonias living in the Russian Federation. A sample of 79 unrelated probands with nondystrophic Thomsen??s and Becker??s myotonias and 44 their relatives has been formed in the Laboratory of DNA Diagnosis of the Medical Genetic Research Center of the Russian Academy of Medical Sciences. Forty CLCN1 gene mutations have been found in a total of 118 chromosomes of 66 probands, including 21 familial and 45 sporadic cases. About half the mutations detected (45%) have been found for the first time; they are not described in the SNP database (ncbi.nlm.nih.gov). The following mutations (substitutions) have been detected in more than one chromosome, accounting for a total of 59.3% of chromosomes with mutations: Gly190Ser (5.9%), c.1437_1450del14 (9.3%), Ala493Glu (5.1%), Thr550Met (3.4%), Tyr686Stop (5.1%), and Arg894Stop (30.5%).  相似文献   

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