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10.1111/j.1582-4934.2010.01187.x http://scihub22266oqcxt.onion/10.1111/j.1582-4934.2010.01187.x C4373368!4373368!21029365     free     free     free Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       J+Cell+Mol+Med 2011 ; 15 (8): 1788-96 Nephropedia Template TP {{tp|p=21029365|t=2011. Fibrotic response in fibroblasts from congenital disorders of glycosylation |pdf=|usr=}}{{21029365}} gab.com Text Fibrotic response in fibroblasts from congenital disorders of glycosylation JO: J Cell Mol Med http://www.kidney.de/pget.php?&tw=1&pmid=21029365 #FOAvip Congenital disorders of glycosylation (CDG) are characterized by a generalized underglycosylation of proteins. CDG is associated with multiple symptoms such as psychomotor retardation, hypotonia, hormonal disturbances, liver fibrosis and coagulopathies. The molecular basis of these symptoms is poorly understood considering the large extent of affected glycoproteins. To better understand the cellular responses to protein underglycosylation in CDG, we have investigated the differences in gene expression between healthy control and CDG fibroblasts by transcriptome comparison. This analysis revealed a strong induction of several genes encoding components of the extracellular matrix, such as collagens, COMP, IGFBP5 and biglycan. The extent of this response was confirmed at the protein level by showing increased production of collagen type-I for example. This fibrotic response of CDG fibroblasts was not paralleled by a differentiation to myofibroblasts and by increased TGF-? signalling. We could show that the addition of recombinant IGFBP5, one of the induced proteins in CDG, to healthy control fibroblasts increased the production of collagen type-I to levels similar to those found in CDG fibroblasts. The fibrotic response identified in CDG fibroblasts may account for the elevated tissue fibrosis, which is often encountered in CDG patients. Twit Text FOAVip Fibrotic response in fibroblasts from congenital disorders of glycosylation ????J Cell Mol Med http://www.kidney.de/pget.php?&tw=1&pmid=21029365 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=21029365 #FOAvip English Wikipedia <ref>{{Cite pmid|21029365}}</ref> Fibrotic response in fibroblasts from congenital disorders of glycosylation #MMPMID21029365 Lecca MR; Maag C; Berger EG; Hennet T J Cell Mol Med 2011[Aug]; 15 (8): 1788-96 PMID21029365show ga Congenital disorders of glycosylation (CDG) are characterized by a generalized underglycosylation of proteins. CDG is associated with multiple symptoms such as psychomotor retardation, hypotonia, hormonal disturbances, liver fibrosis and coagulopathies. The molecular basis of these symptoms is poorly understood considering the large extent of affected glycoproteins. To better understand the cellular responses to protein underglycosylation in CDG, we have investigated the differences in gene expression between healthy control and CDG fibroblasts by transcriptome comparison. This analysis revealed a strong induction of several genes encoding components of the extracellular matrix, such as collagens, COMP, IGFBP5 and biglycan. The extent of this response was confirmed at the protein level by showing increased production of collagen type-I for example. This fibrotic response of CDG fibroblasts was not paralleled by a differentiation to myofibroblasts and by increased TGF-? signalling. We could show that the addition of recombinant IGFBP5, one of the induced proteins in CDG, to healthy control fibroblasts increased the production of collagen type-I to levels similar to those found in CDG fibroblasts. The fibrotic response identified in CDG fibroblasts may account for the elevated tissue fibrosis, which is often encountered in CDG patients. äFibrotic response in fibroblasts from congenital disorders of glycosyl(epmc).pdf DeepDyve Pubget Overpricing