574 research outputs found

    Glycogenin is Dispensable for Glycogen Synthesis in Human Muscle, and Glycogenin Deficiency Causes Polyglucosan Storage

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    Glycogenin is considered to be an essential primer for glycogen biosynthesis. Nevertheless, patients with glycogenin-1 deficiency due to biallelic GYG1 (NM_004130.3) mutations can store glycogen in muscle. Glycogenin-2 has been suggested as an alternative primer for glycogen synthesis in patients with glycogenin-1 deficiency. OBJECTIVE: The objective of this article is to investigate the importance of glycogenin-1 and glycogenin-2 for glycogen synthesis in skeletal and cardiac muscle. DESIGN, SETTING, AND PATIENTS: Glycogenin-1 and glycogenin-2 expression was analyzed by Western blot, mass spectrometry, and immunohistochemistry in liver, heart, and skeletal muscle from controls and in skeletal and cardiac muscle from patients with glycogenin-1 deficiency. RESULTS: Glycogenin-1 and glycogenin-2 both were found to be expressed in the liver, but only glycogenin-1 was identified in heart and skeletal muscle from controls. In patients with truncating GYG1 mutations, neither glycogenin-1 nor glycogenin-2 was expressed in skeletal muscle. However, nonfunctional glycogenin-1 but not glycogenin-2 was identified in cardiac muscle from patients with cardiomyopathy due to GYG1 missense mutations. By immunohistochemistry, the mutated glycogenin-1 colocalized with the storage of glycogen and polyglucosan in cardiomyocytes. CONCLUSIONS: Glycogen can be synthesized in the absence of glycogenin, and glycogenin-1 deficiency is not compensated for by upregulation of functional glycogenin-2. Absence of glycogenin-1 leads to the focal accumulation of glycogen and polyglucosan in skeletal muscle fibers. Expression of mutated glycogenin-1 in the heart is deleterious, and it leads to storage of abnormal glycogen and cardiomyopathy

    Effects of particle geometry and chemical accelerator on strength properties of rattan–cement composites

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    Rattan-cement composites were manufactured using ‘as-received’ rattan particles and those retained on 0.60mm and 0.85mm sieves. Cement: rattan mixing ratio (by weight) was 1: 0.11. Calcium chloride (CaCl2) was incorporated at two levels, i.e., 0% (control) and 3% by weight of cement. Findings indicated that ‘as received’ particles produced denser, stronger and stiffer  composites than the two other particles investigated. Addition of Cacl2 resulted in an increase of between 1.6% and 9.9% in density and had significant effects on Modulus of Elasticity, Modulus of Rupture, and the compressive strength of the composites. Inverse relationships were observed between particle size and product density.Keywords: rattan-cement composite, calcium chloride, strength propertie

    Technology and market opportunities in fiber cement composites for small scale enterprises in Nigeria

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    Solving problems of unemployment and developing new materials for buildings requires moving beyond the traditional approaches to more economical, environmentally benign performance models and design properties. New approaches should develop models based on the literature and a full understanding of the root causes of failure, derived from careful failure analysis. One of the main drivers of the development efforts in fibre based composites is the trend towards greater environmental awareness and the health hazards associated with the utilization of asbestos fibres. The key to effectively developing marketable fibre cement composites for use as building materials that can be used for construction of safe and affordable structures is to identify the research and  development and market needs for such products. This paper looks into the empirical production and market opportunities in terms of product performance accruable to locally manufactured fibre cement ceiling boards.KEY WORDS: Fibre Cement Boards, Production, Market Needs, Product Performanc

    Heteroglossia: Bakhtinian dialogism within a play's monologue

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    This study tries to expand the richness of Bakhtin's theory of novel by showing the reader that its thorough features could be traced back in a play rather than a novel, considering it more than what is usually the basis of "historical poetics" mainly in the form of a novel accentuating the constitution of a social ideology besides an individual one while gesturing dialogically in the interaction between representation in its textual form and particularities of its proper probable forces in their socio-historical stratifications within notions such as dialogism, intertextuality, heteroglossia and polyphony. To do so a successful Irish play of exuberance is invited to be served by a thinker from the past Soviet. Since the references are written in an artistic language, a language near to a poetic one tries to tinker rationality to irrationality. In the light of O’Halloran's eccentric nostalgia which tries to handle a play all in all monologically from the voice of just a single character, one may seem to be listening to the symphony of Bakhtin's polyphonic heteroglossia stratified within the architectonics of both authors' interillumination

    Examining the Dimensions of Big Data Privacy (Block Chain Solution for Privacy Protection)

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    Purpose: In the past, big data was the concern of governments and large commercial industries, and so these organizations had infrastructures separate from the public network to store and process their data. But today, big data is easily accessible to everyone through cloud infrastructure. In recent years, with the explosive development of the Internet, data storage and data processing technologies, privacy has been one of the major concerns in data mining Methodology: In this paper, after examining the dimensions and key components of flax data, privacy in big data; Blockchain solution for privacy protection is examined. Findings: This topic provides new opportunities for researchers in knowledge processing tasks. However, these opportunities often bring challenges as well. Originality/Value: Big Data means a very large database that can be structured or unstructured. Big data is a database that is constantly getting bigger with the increase of information. Also with the rapid advancement of the Internet, data storage and data processing technologies, big data privacy has attracted a lot of attention. Before applying big data technology to mass applications and applications, a basic issue called privacy must be explored

    Vpliv velikosti bukovega iverja, uporabljenega v zunanjem sloju na upogibno trdnost trislojne iverne plošče

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    Znano je, da vrsta lesa in velikost iverja vplivata na upogibno trdnost trislojnih ivernih plošč. Namen posebej zasnovane raziskave je bil ugotoviti, kako velikost bukovega (Fagus Sylvatica L.) iverja vpliva na upogibno trdnost trislojnih ivernih plošč, če je velikost bukovega iverja spremenjena samo v zunanjem sloju. Uporabljeno je bilo bukovo iverje petih različnih velikosti. Iverje je bilo oblepljeno z UF lepilom. Rezultati meritev so pokazali, da upogibna trdnost ivernih plošč narašča z naraščanjem specifične površine iverja, faktorja zgostitve zunanjega sloja in prostorninske mase zunanjega sloja, medtem ko z naraščanjem oblepljenosti upogibna trdnost pada.It is well known that wood species and particle size used influence the bending strength of the three-layer particleboard. The purpose of this research was to determine how the size of beech (Fagus sylvatica L.) particlesinfluences the bending strength of the three-layer particleboard, where size of particles used was altered in surface layer only. Beech wood particles of five different sizes were used and blended with UF adhesive. The results show that the bending strength of boards increases with increasing particle specific surface area, increasing surface layer compaction ratio and surface layer density, while bending strength decreases with increasing surface covered with adhesive

    Philosophic (in)felicity: protean narrativity

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    Philosophic contingency embedded in James Joyce's avant-garde novel, ranging from Aristotelian notion of mimesis to the possibility of a Marxian reading, stemming from Hegel's dialectic, added to the linguistic pragmatics, pave the way to focus on the process of realization and mental performance by a leading contemporary philosopher of analytic philosophy, J.L. Austin, as an epistemological triggering in the course of implication through a narrative, here Proteus, teleologically speaking, resulting a meta-utterance in a broader scale, much far from constative type, a metaphorical narration, elaborating on cultural agency, while paraphrasing the language-based relativity of collective identity and complexity of Austin’s speech act theory, in terms of (in)felicitous conditions, rooted in the success of the communicative intention of the narrator, here Stephen Dedalus, maybe the most wonderful advanced guard of narratology in interior monologue

    In between

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    The thesis explores the space between anticipation and experience; The journey from knowing to recognizing the unknown. Series of drawings, models and videos try to illustrate the space in between

    Effect of Fluoride Gel on Microhardness of Flowable Composites: An In Vitro Study

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    Objective: Adequate knowledge about restorative materials particularly flowable composite resins with favorable physical properties like optimal microhardness and resistance to environmental factors is a big concern for many dentists. Flowable composites have several applications for fissure sealant therapy and preventive resin restorations (PRR); which are common treatments in pediatric dentistry.  Acidulated Phosphor Fluoride (APF) topical gels can cause surface degradation and  weight loss in composite resins and decrease their wear resistance. This study aimed to assess the effect of APF gel on the microhardness of flowable composites.Methods: This experimental in vitro study was conducted on 60 specimens measuring 2mm in thickness and 6mm in diameter fabricated of Tetric N-Flow (Ivoclar-Vivadent), PermaFlo  (Ultradent) and Denfil (Vericom) flowable composites (n=20 for each group). Specimens were stored in artificial saliva at room temperature for one week. The 20 specimens in each group were randomly divided into 2 subgroups of test and control (n=10). Microhardness was measured using Vickers microhardness tester. In the control groups, 3 indentations were made on each disc on the periphery of a circle with at least 1mm distance from one another and from the specimen margin.  The mean microhardness value was then calculated. The mean of all measurements was calculated  as well. Test specimens were subjected to (Sultan) 1.23% APF gel for 4min and then rinsed and air- dried. Their microhardness was measured again as described above. Two-way ANOVA was used to compare the effect of APF gel and type of composite on the microhardness of different flowable composites.Results: Based on the results, the microhardness of Denfil, PermaFlo and Tetric N-Flow was 16.5 (1.32), 37.36 (2.13) and 20.39 (0.52) before and 16.46 (2.20), 35.04 (2.43) and 19.13 (2.20) after theapplication of APF gel, respectively. The difference between the before and after values for each composite was significant (p=0.193) while the difference between different composites was statistically significant in this regard (p<0.001).Conclusion: Exposure of Denfil, PermaFlo and Tetric N-Flow flowable composites to APF gel for 4min had no effect on their microhardness
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