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10.1073/pnas.1620139114 http://scihub22266oqcxt.onion/10.1073/pnas.1620139114 C5278481!5278481!28069966     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       Proc+Natl+Acad+Sci+U+S+A 2017 ; 114 (4): E514-23 Nephropedia Template TP {{tp|p=28069966|t=2017. Inherited human IRAK-1 deficiency selectively impairs TLR signaling in fibroblasts |pdf=|usr=}}{{28069966}} gab.com Text Inherited human IRAK-1 deficiency selectively impairs TLR signaling in fibroblasts JO: Proc Natl Acad Sci U S A http://www.kidney.de/pget.php?&tw=1&pmid=28069966 #FOAvip We report the discovery of complete human interleukin-1 receptor (IL-1R)-associated kinase 1 (IRAK-1) deficiency resulting from a de novo Xq28 microdeletion encompassing MECP2 and IRAK1 in a boy. Like many boys with MECP2 defects, this patient died very early. IRAK-1 is a component of the Toll-like receptor (TLR)/IL-1R (TIR) signaling pathway. Unlike patients with autosomal-recessive complete deficiency of MyD88 or IRAK-4, two other components of the TIR pathway, this patient presented no invasive bacterial infections. We analyzed the impact of human IRAK-1 deficiency in fibroblasts and leukocytes. The role of IRAK-1 in signaling downstream from IL-1R and TLRs differed according to cell type. These findings reveal similarities and differences in the role of IRAK-1 in the TLR and IL-1R pathways between mice and humans. Twit Text FOAVip Inherited human IRAK-1 deficiency selectively impairs TLR signaling in fibroblasts ????Proc Natl Acad Sci U S A http://www.kidney.de/pget.php?&tw=1&pmid=28069966 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28069966 #FOAvip English Wikipedia <ref>{{Cite pmid|28069966}}</ref> Inherited human IRAK-1 deficiency selectively impairs TLR signaling in fibroblasts #MMPMID28069966 Della Mina E; Borghesi A; Zhou H; Bougarn S; Boughorbel S; Israel L; Meloni I; Chrabieh M; Ling Y; Itan Y; Renieri A; Mazzucchelli I; Basso S; Pavone P; Falsaperla R; Ciccone R; Cerbo RM; Stronati M; Picard C; Zuffardi O; Abel L; Chaussabel D; Marr N; Li X; Casanova JL; Puel A Proc Natl Acad Sci U S A 2017[Jan]; 114 (4): E514-23 PMID28069966show ga We report the discovery of complete human interleukin-1 receptor (IL-1R)-associated kinase 1 (IRAK-1) deficiency resulting from a de novo Xq28 microdeletion encompassing MECP2 and IRAK1 in a boy. Like many boys with MECP2 defects, this patient died very early. IRAK-1 is a component of the Toll-like receptor (TLR)/IL-1R (TIR) signaling pathway. Unlike patients with autosomal-recessive complete deficiency of MyD88 or IRAK-4, two other components of the TIR pathway, this patient presented no invasive bacterial infections. We analyzed the impact of human IRAK-1 deficiency in fibroblasts and leukocytes. The role of IRAK-1 in signaling downstream from IL-1R and TLRs differed according to cell type. These findings reveal similarities and differences in the role of IRAK-1 in the TLR and IL-1R pathways between mice and humans. äInherited human IRAK-1 deficiency selectively impairs TLR signaling in(epmc).pdf DeepDyve Pubget Overpricing