Use my Search Websuite to scan PubMed, PMCentral, Journal Hosts and Journal Archives, FullText. Kick-your-searchterm to multiple Engines kick-your-query now !>

A dictionary by aggregated review articles of nephrology, medicine and the life sciences Your one-stop-run pathway from word to the immediate pdf of peer-reviewed on-topic knowledge.

10.1073/pnas.96.11.6423 http://scihub22266oqcxt.onion/10.1073/pnas.96.11.6423 10339603!26897!10339603     free     free     free Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 235.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Proc+Natl+Acad+Sci+U+S+A 1999 ; 96 (11): 6423-7 Nephropedia Template TP {{tp|p=10339603|t=1999. Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice |pdf=|usr=}}{{10339603}} gab.com Text Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice JO: Proc Natl Acad Sci U S A http://www.kidney.de/pget.php?&tw=1&pmid=10339603 #FOAvip Fabry disease is an X-linked metabolic disorder caused by a deficiency of alpha-galactosidase A (alpha-Gal A). The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide (Gb3). In patients with this disorder, glycolipid deposition in endothelial cells leads to renal failure and cardiac and cerebrovascular disease. Recently, we generated alpha-Gal A gene knockout mouse lines and described the phenotype of 10-week-old mice. In the present study, we characterize the progression of the disease with aging and explore the effects of bone marrow transplantation (BMT) on the phenotype. Histopathological analysis of alpha-Gal A -/0 mice revealed subclinical lesions in the Kupffer cells in the liver and macrophages in the skin with no gross lesions in the endothelial cells. Gb3 accumulation and pathological lesions in the affected organs increased with age. Treatment with BMT from the wild-type mice resulted in the clearance of accumulated Gb3 in the liver, spleen, and heart with concomitant elevation of alpha-Gal A activity. These findings suggest that BMT may have a potential role in the management of patients with Fabry disease. Twit Text FOAVip Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice ????Proc Natl Acad Sci U S A http://www.kidney.de/pget.php?&tw=1&pmid=10339603 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=10339603 #FOAvip English Wikipedia <ref>{{Cite pmid|10339603}}</ref> Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice #MMPMID10339603 Ohshima T; Schiffmann R; Murray GJ; Kopp J; Quirk JM; Stahl S; Chan CC; Zerfas P; Tao-Cheng JH; Ward JM; Brady RO; Kulkarni AB Proc Natl Acad Sci U S A 1999[May]; 96 (11): 6423-7 PMID10339603show ga Fabry disease is an X-linked metabolic disorder caused by a deficiency of alpha-galactosidase A (alpha-Gal A). The enzyme defect leads to the systemic accumulation of glycosphingolipids with alpha-galactosyl moieties consisting predominantly of globotriaosylceramide (Gb3). In patients with this disorder, glycolipid deposition in endothelial cells leads to renal failure and cardiac and cerebrovascular disease. Recently, we generated alpha-Gal A gene knockout mouse lines and described the phenotype of 10-week-old mice. In the present study, we characterize the progression of the disease with aging and explore the effects of bone marrow transplantation (BMT) on the phenotype. Histopathological analysis of alpha-Gal A -/0 mice revealed subclinical lesions in the Kupffer cells in the liver and macrophages in the skin with no gross lesions in the endothelial cells. Gb3 accumulation and pathological lesions in the affected organs increased with age. Treatment with BMT from the wild-type mice resulted in the clearance of accumulated Gb3 in the liver, spleen, and heart with concomitant elevation of alpha-Gal A activity. These findings suggest that BMT may have a potential role in the management of patients with Fabry disease. |*Bone Marrow Transplantation[MESH] |*Fabry Disease/pathology/*physiopathology/*therapy[MESH] |Aging/*physiology[MESH] |Animals[MESH] |Crosses, Genetic[MESH] |Genotype[MESH] |Glycosphingolipids/metabolism[MESH] |Heart/growth & development[MESH] |Liver/growth & development/pathology/ultrastructure[MESH] |Male[MESH] |Mice[MESH] |Mice, Inbred C57BL[MESH] |Mice, Inbred Strains[MESH] |Mice, Knockout[MESH] |Myocardium/pathology/ultrastructure[MESH] |Phenotype[MESH] |Polymerase Chain Reaction[MESH] |Skin/growth & development/pathology/ultrastructure[MESH]Aging accentuates and bone marrow transplantation ameliorates metaboli(epmc).pdf DeepDyve Pubget Overpricing