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51.
Claudio Bruno Denise Cassandrini Fabiana Fattori Marina Pedemonte Chiara Fiorillo Giorgia Brigati Giacomo Brisca Carlo Minetti Filippo M. Santorelli 《Biochemical and biophysical research communications》2011,(4):518
We report an 11-year-old boy with exercise-related myopathy, and a novel mutation m.5669G>A in the mitochondrial tRNA Asparagine gene (mt-tRNAAsn, MTTN). Muscle biopsy studies showed COX-negative, SDH-positive fibers at histochemistry and biochemical defects of oxidative metabolism. The m.5669G>A mutation was present only in patient’s muscle resulting in the first muscle-specific MTTN mutation. Mt-tRNAAsn steady-state levels and in silico predictions supported the pathogenicity of this mutation. A mitochondrial myopathy should be considered in the differential diagnosis of exercise intolerance in children. 相似文献
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Pauline?AaltenEmail author Inez?HGB?Ramakers Geert?Jan?Biessels Peter?Paul?de Deyn Huiberdina?L?Koek Marcel?GM?OldeRikkert Ania?M?Oleksik Edo?Richard Lieke?L?Smits John?C?van Swieten Laura?K?Teune Aad?van der Lugt Frederik?Barkhof Charlotte?E?Teunissen Nico?Rozendaal Frans?RJ?Verhey Wiesje?M?van der Flier 《BMC neurology》2014,14(1):254
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Aster SD Graham DW Kharbanda D Patel G Ponpipom M Santorelli GM Szymonifka MJ Mundt SS Shah K Springer MS Thieringer R Hermanowski-Vosatka A Wright SD Xiao J Zokian H Balkovec JM 《Bioorganic & medicinal chemistry letters》2008,18(9):2799-2804
3-Aryl-5-phenyl-(1,2,4)-triazoles were identified as selective inhibitors of 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1). They are active in both in vitro and an in vivo mouse pharmacodynamic (PD) model. The synthesis and structure activity relationships are presented. 相似文献
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Background
Listeria monocytogenesis a well-characterized food-borne pathogen that infects pregnant women and immunocompromised individuals. Listeriolysin O (LLO) is the major virulence factor of the pathogen and is often used as a diagnostic marker for detection of L. monocytogenes. In addition, LLO represents a potent antigen driving T cell-mediated immunity during infection. In the present work, Lactococcus lactisNZ9000 was used as an expression host to hyper-produce LLO under inducible conditions using the NICE (NIsin Controlled Expression) system. We created a modified pNZ8048 vector encoding a six-His-tagged LLO downstream of the strong inducible PnisA promoter. 相似文献58.
Rick Brouwer Wilma TM Vree Egberts Gerald JD Hengstman Reinout Raijmakers Baziel GM van Engelen Hans Peter Seelig Manfred Renz Rudolf Mierau Ekkehard Genth Ger JM Pruijn Walther J van Venrooij 《Arthritis research & therapy》2001,4(2):1-5
The autoantigenic polymyositis/scleroderma (PM/Scl) complex was recently shown to be the human homologue of the yeast exosome, which is an RNA-processing complex. Our aim was to assess whether, in addition to targeting the known autoantigens PM/Scl-100 and PM/Scl-75, autoantibodies also target recently identified components of the PM/Scl complex. The prevalence of autoantibodies directed to six novel human exosome components (hRrp4p, hRrp40p, hRrp41p, hRrp42p, hRrp46p, hCsl4p) was determined in sera from patients with idiopathic inflammatory myopathy (n = 48), scleroderma (n = 11), or the PM/Scl overlap syndrome (n = 10). The sera were analyzed by enzyme-linked immunosorbent assays and western blotting using the affinity-purified recombinant proteins. Our results show that each human exosome component is recognized by autoantibodies. The hRrp4p and hRrp42p components were most frequently targeted. The presence of autoantibodies directed to the novel components of the human exosome was correlated with the presence of the anti-PM/Scl-100 autoantibody in the sera of patients with idiopathic inflammatory myopathy (IIM), as was previously found for the anti-PM/Scl-75 autoantibody. Other clear associations between autoantibody activities were not found. These results further support the conception that the autoimmune response may initially be directed to PM/Scl-100, whereas intermolecular epitope spreading may have caused the autoantibody response directed to the associated components. 相似文献
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Acid α-glucosidase (GAA) is a lysosomal enzyme that hydrolyzes glycogen to glucose. Deficiency of GAA causes Pompe disease. Mammalian GAA is synthesized as a precursor of ~ 110,000 Da that is N-glycosylated and targeted to the lysosome via the M6P receptors. In the lysosome, human GAA is sequentially processed by proteases to polypeptides of 76-, 19.4-, and 3.9-kDa that remain associated. Further cleavage between R200 and A204 inefficiently converts the 76-kDa polypeptide to the mature 70-kDa form with an additional 10.4-kDa polypeptide. GAA maturation increases its affinity for glycogen by 7-10 fold. In contrast to human GAA, processing of bovine and hamster GAA to the 70-kDa form is more rapid. A comparison of sequences surrounding the cleavage site revealed human GAA contains histidine at 201 while other species contain hydrophobic amino acids at position 201 in the otherwise conserved sequence. Recombinant human GAA (rhGAA) containing the H201L substitution was expressed in 293 T cells by transfection. Pulse chase experiments in 293 T cells expressing rhGAA with or without the H201L substitution revealed rapid processing of rhGAAH201L but not rhGAAWT to the 70-kDa form. Similarly, when GAA precursor was endocytosed by human Pompe fibroblasts rhGAAH201L but not rhGAAWT was rapidly converted to the 70-kDa mature GAA. These studies indicate that the amino acid at position 201 influences the rate of conversion of 76-kDa GAA to 70-kDa GAA. The GAA sequence rather than the lysosomal protease environment explains the predominance of the 76-kDa form in human tissues. 相似文献
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Suzanne Arends Anneke Spoorenberg Pieternella M Houtman Martha K Leijsma Reinhard Bos Cees GM Kallenberg Henk Groen Elisabeth Brouwer Eveline van der Veer 《Arthritis research & therapy》2012,14(2):1-10