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10.1042/BJ20130825 http://scihub22266oqcxt.onion/10.1042/BJ20130825 24094090!4160053!24094090     free     free     free Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Biochem+J 2013 ; 456 (3): 373-83 Nephropedia Template TP {{tp|p=24094090|t=2013. A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis |pdf=|usr=}}{{24094090}} gab.com Text A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis JO: Biochem J http://www.kidney.de/pget.php?&tw=1&pmid=24094090 #FOAvip Fabry disease is a lysosomal storage disorder in which neutral glycosphingolipids, predominantly Gb3 (globotriaosylceramide), accumulate due to deficient alpha-Gal A (alpha-galactosidase A) activity. The GLAko (alpha-Gal A-knockout) mouse has been used as a model for Fabry disease, but it does not have any symptomatic abnormalities. In the present study, we generated a symptomatic mouse model (G3Stg/GLAko) by cross-breeding GLAko mice with transgenic mice expressing human Gb3 synthase. G3Stg/GLAko mice had high Gb3 levels in major organs, and their serum Gb3 level at 5-25 weeks of age was 6-10-fold higher than that in GLAko mice of the same age. G3Stg/GLAko mice showed progressive renal impairment, with albuminuria at 3 weeks of age, decreased urine osmolality at 5 weeks, polyuria at 10 weeks and increased blood urea nitrogen at 15 weeks. The urine volume and urinary albumin concentration were significantly reduced in the G3Stg/GLAko mice when human recombinant alpha-Gal A was administered intravenously. These data suggest that Gb3 accumulation is a primary pathogenic factor in the symptomatic phenotype of G3Stg/GLAko mice, and that this mouse line is suitable for studying the pathogenesis of Fabry disease and for preclinical studies of candidate therapies. Twit Text FOAVip A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis ????Biochem J http://www.kidney.de/pget.php?&tw=1&pmid=24094090 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24094090 #FOAvip English Wikipedia <ref>{{Cite pmid|24094090}}</ref> A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis #MMPMID24094090 Taguchi A; Maruyama H; Nameta M; Yamamoto T; Matsuda J; Kulkarni AB; Yoshioka H; Ishii S Biochem J 2013[Dec]; 456 (3): 373-83 PMID24094090show ga Fabry disease is a lysosomal storage disorder in which neutral glycosphingolipids, predominantly Gb3 (globotriaosylceramide), accumulate due to deficient alpha-Gal A (alpha-galactosidase A) activity. The GLAko (alpha-Gal A-knockout) mouse has been used as a model for Fabry disease, but it does not have any symptomatic abnormalities. In the present study, we generated a symptomatic mouse model (G3Stg/GLAko) by cross-breeding GLAko mice with transgenic mice expressing human Gb3 synthase. G3Stg/GLAko mice had high Gb3 levels in major organs, and their serum Gb3 level at 5-25 weeks of age was 6-10-fold higher than that in GLAko mice of the same age. G3Stg/GLAko mice showed progressive renal impairment, with albuminuria at 3 weeks of age, decreased urine osmolality at 5 weeks, polyuria at 10 weeks and increased blood urea nitrogen at 15 weeks. The urine volume and urinary albumin concentration were significantly reduced in the G3Stg/GLAko mice when human recombinant alpha-Gal A was administered intravenously. These data suggest that Gb3 accumulation is a primary pathogenic factor in the symptomatic phenotype of G3Stg/GLAko mice, and that this mouse line is suitable for studying the pathogenesis of Fabry disease and for preclinical studies of candidate therapies. |Albuminuria/drug therapy/genetics/metabolism/pathology[MESH] |Animals[MESH] |Disease Models, Animal[MESH] |Fabry Disease/drug therapy/genetics/*metabolism/pathology[MESH] |Galactosyltransferases/genetics/*metabolism[MESH] |Humans[MESH] |Mice[MESH] |Mice, Transgenic[MESH] |Trihexosylceramides/*biosynthesis/genetics[MESH]A symptomatic Fabry disease mouse model generated by inducing globotr(epmc).pdf DeepDyve Pubget Overpricing