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排序方式: 共有847条查询结果,搜索用时 15 毫秒
841.
Roberto Tiribuzi Francesco D'Angelo Anna C. Berardi Sabata Martino Aldo Orlacchio 《Cell biochemistry and function》2012,30(1):61-68
In an attempt to investigate whether the genetic defect in the HEXA and HEXB genes (which causes the absence of the lysosomal β‐N‐acetyl‐hexosaminidase), are related to the wide inflammation in GM2 gangliosidoses (Tay‐Sachs and Sandhoff disease), we have chosen the dendritic cells (DCs) as a study model. Using the RNA interference approach, we generated an in vitro model of HEXs knock‐down immunogenic DCs (i‐DCs) from CD34+‐haemopoietic stem cells (CD34+‐HSCs), thus mimicking the Tay‐Sachs (HEXA?/?) and Sandhoff (HEXB?/?) cells. We showed that the absence of β‐N‐acetyl‐hexosaminidase activity does not alter the differentiation of i‐DCs from HSCs, but it is critical for the activation of CD4+T cells because knock‐down of HEXA or HEXB gene causes a loss of function of i‐DCs. Notably, the silencing of the HEXA gene had a stronger immune inhibitory effect, thereby indicating a major involvement of β‐N‐acetyl‐hexosaminidase A isoenzyme within this mechanism. Copyright © 2011 John Wiley & Sons, Ltd. 相似文献
842.
Massimo Aureli Maura Samarani Nicoletta Loberto Elena Chiricozzi Laura Mauri Sara Grassi Domitilla Schiumarini Alessandro Prinetti Sandro Sonnino 《Glycoconjugate journal》2018,35(4):397-402
Sphingolipid metabolism is an intricate network of several interdependent and co-regulated pathways. In addition to the mainstream biosynthetic and catabolic pathways, several processes, even if less important in contributing to the final tissue sphingolipid composition from the quantitative point of view, might become relevant when sphingolipid metabolism is for any reason dysregulated and concur to the onset of neuronal pathologies. The main subcellular sites involved in the mainstream metabolic pathway are represented by the Golgi apparatus (for the biosynthesis) and by the lysosomes (for catabolism). On the other hand, the minor collateral pathways are associated with the plasma membrane and membranes of other organelles, and likely play important roles in the local regulation of membrane dynamics and contribute to maintain a perfect membrane organization functional to the physiology of the cell. In this review, we will consider few aspects of the sphingolipid metabolic pathway depending by the dynamic of the membranes that seems to become relevant in neurodegenerative diseases. 相似文献
843.
Albanese Valeria Kuan Michela Accorsi Pier Attilio Berardi Roberta Marliani Giovanna 《Primates; journal of primatology》2021,62(4):585-593
Primates - Long-tailed macaques are highly social primates that are commonly used in biomedical research as animal models. The aim of this study was to evaluate the effects of different kinds of... 相似文献
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846.
Francesca Zambianchi Stefano Raimondi Piero Pasta Giacomo Carrea Nicoletta Gaggero John M. Woodley 《Journal of Molecular Catalysis .B, Enzymatic》2004,31(4-6):165-171
Both whole cells of recombinant Escherichia coli TOP10, overexpressing cyclohexanone monooxygenase (CHMO) and isolated cyclohexanone monooxygenase, were used to carry out the enantioselective oxidation of 1,3-dithiane (1) to (R)-1,3-dithiane-1-oxide (2). The two biocatalysts were evaluated under various experimental conditions (e.g., shaken flask or bioreactor; non-bound or resin-adsorbed substrate; different substrate concentrations) in terms of volumetric productivity and enantioselectivity. While productivity was similar in the two cases (up to 0.58 g L−1 h−1), the optical purity of the product was much higher with the isolated enzyme (up to 98% e.e.) than with the whole cell biocatalyst (up to 85% e.e.). 相似文献
847.
Veronique van den Berghe Elke Stappers Bram Vandesande Jordane Dimidschstein Roel Kroes Annick Francis Andrea Conidi Flore Lesage Ruben Dries Silvia Cazzola Geert Berx Nicoletta Kessaris Pierre Vanderhaeghen Wilfred van IJcken Frank G. Grosveld Steven Goossens Jody J. Haigh Gord Fishell Eve Seuntjens 《Neuron》2013,77(1):70-82