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10.1681/ASN.2015040348 http://scihub22266oqcxt.onion/10.1681/ASN.2015040348 C4884110!4884110 !26471127     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 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 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26471127 .jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       J+Am+Soc+Nephrol 2016 ; 27 (6 ): 1665-77 Nephropedia Template TP {{tp|p=26471127 |t=2016. A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H |pdf=|usr=}}{{26471127 }} gab.com Text A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H JO: J Am Soc Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=26471127 #FOAvip C3 glomerulopathy is a recently described form of CKD. C3GN is a subtype of C3 glomerulopathy characterized by predominant C3 deposits in the glomeruli and is commonly the result of acquired or genetic abnormalities in the alternative pathway (AP) of the complement system. We identified and characterized the first mutation of the C3 gene (p. I734T) in two related individuals diagnosed with C3GN. Immunofluorescence and electron microscopy studies showed C3 deposits in the subendothelial space, associated with unusual deposits located near the complement receptor 1 (CR1)-expressing podocytes. In vitro, this C3 mutation exhibited decreased binding to CR1, resulting in less CR1-dependent cleavage of C3b by factor 1. Both patients had normal plasma C3 levels, and the mutant C3 interacted with factor B comparably to wild-type (WT) C3 to form a C3 convertase. Binding of mutant C3 to factor H was normal, but mutant C3 was less efficiently cleaved by factor I in the presence of factor H, leading to enhanced C3 fragment deposition on glomerular cells. In conclusion, our results reveal that a CR1 functional deficiency is a mechanism of intraglomerular AP dysregulation and could influence the localization of the glomerular C3 deposits. Twit Text FOAVip A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H ????J Am Soc Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=26471127 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26471127 #FOAvip English Wikipedia <ref>{{Cite pmid|26471127 }}</ref> A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H #MMPMID26471127 Chauvet S ; Roumenina LT ; Bruneau S ; Marinozzi MC ; Rybkine T ; Schramm EC ; Java A ; Atkinson JP ; Aldigier JC ; Bridoux F ; Touchard G ; Fremeaux-Bacchi V J Am Soc Nephrol 2016[Jun]; 27 (6 ): 1665-77 PMID26471127 show ga C3 glomerulopathy is a recently described form of CKD. C3GN is a subtype of C3 glomerulopathy characterized by predominant C3 deposits in the glomeruli and is commonly the result of acquired or genetic abnormalities in the alternative pathway (AP) of the complement system. We identified and characterized the first mutation of the C3 gene (p. I734T) in two related individuals diagnosed with C3GN. Immunofluorescence and electron microscopy studies showed C3 deposits in the subendothelial space, associated with unusual deposits located near the complement receptor 1 (CR1)-expressing podocytes. In vitro, this C3 mutation exhibited decreased binding to CR1, resulting in less CR1-dependent cleavage of C3b by factor 1. Both patients had normal plasma C3 levels, and the mutant C3 interacted with factor B comparably to wild-type (WT) C3 to form a C3 convertase. Binding of mutant C3 to factor H was normal, but mutant C3 was less efficiently cleaved by factor I in the presence of factor H, leading to enhanced C3 fragment deposition on glomerular cells. In conclusion, our results reveal that a CR1 functional deficiency is a mechanism of intraglomerular AP dysregulation and could influence the localization of the glomerular C3 deposits. |Adult [MESH] |Complement C3/*genetics [MESH] |Complement Factor H/genetics [MESH] |Gene Expression Regulation [MESH] |Glomerulonephritis, Membranoproliferative/*genetics [MESH] |Humans [MESH] |Male [MESH] |Middle Aged [MESH] |Pedigree [MESH]A Familial C3GN Secondary to Defective C3 Regulation by Complement Rec(epmc).pdf DeepDyve Pubget Overpricing