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10.1007/s00467-013-2652-z http://scihub22266oqcxt.onion/10.1007/s00467-013-2652-z C4018427!4018427!24217784     free     free     free Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Pediatr+Nephrol 2014 ; 29 (12): 2253-61 Nephropedia Template TP {{tp|p=24217784|t=2014. Lysosome dysfunction in the pathogenesis of kidney diseases |pdf=|usr=}}{{24217784}} gab.com Text Lysosome dysfunction in the pathogenesis of kidney diseases JO: Pediatr Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=24217784 #FOAvip The lysosome, an organelle central to macromolecule degradation and recycling, plays a pivotal role in normal cell processes, ranging from autophagy to redox regulation. Not surprisingly, lysosomes are an integral part of the renal epithelial molecular machinery that facilitates normal renal physiology. Two inherited diseases that manifest as kidney dysfunction are Fabry?s disease and cystinosis, each of which is caused by a primary biochemical defect at the lysosome resulting from loss of function mutations in genes that encode lysosomal proteins. The functions of the lysosomes in the kidney and how lysosomal dysfunction might contribute to Fabry?s disease and cystinosis are discussed. Unlike most other pediatric renal diseases, therapies are available for Fabry?s disease and cystinosis, but require early diagnosis. Recent analysis of ceroid neuronal lipofuscinosis type 3 (Cln3) null mice, a mouse model of lysosomal disease that is primarily associated with neurological deficits, revealed renal functional abnormalities. As current and future therapeutics increase the life-span of those suffering from diseases like neuronal ceroid lipofuscinosis, it remains a distinct possibility that many more lysosomal disorders that primarily manifest as infant and juvenile neurodegenerative diseases may also include renal disease phenotypes. Twit Text FOAVip Lysosome dysfunction in the pathogenesis of kidney diseases ????Pediatr Nephrol http://www.kidney.de/pget.php?&tw=1&pmid=24217784 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24217784 #FOAvip English Wikipedia <ref>{{Cite pmid|24217784}}</ref> Lysosome dysfunction in the pathogenesis of kidney diseases #MMPMID24217784 Surendran K; Vitiello SP; Pearce DA Pediatr Nephrol 2014[Dec]; 29 (12): 2253-61 PMID24217784show ga The lysosome, an organelle central to macromolecule degradation and recycling, plays a pivotal role in normal cell processes, ranging from autophagy to redox regulation. Not surprisingly, lysosomes are an integral part of the renal epithelial molecular machinery that facilitates normal renal physiology. Two inherited diseases that manifest as kidney dysfunction are Fabry?s disease and cystinosis, each of which is caused by a primary biochemical defect at the lysosome resulting from loss of function mutations in genes that encode lysosomal proteins. The functions of the lysosomes in the kidney and how lysosomal dysfunction might contribute to Fabry?s disease and cystinosis are discussed. Unlike most other pediatric renal diseases, therapies are available for Fabry?s disease and cystinosis, but require early diagnosis. Recent analysis of ceroid neuronal lipofuscinosis type 3 (Cln3) null mice, a mouse model of lysosomal disease that is primarily associated with neurological deficits, revealed renal functional abnormalities. As current and future therapeutics increase the life-span of those suffering from diseases like neuronal ceroid lipofuscinosis, it remains a distinct possibility that many more lysosomal disorders that primarily manifest as infant and juvenile neurodegenerative diseases may also include renal disease phenotypes. äLysosome dysfunction in the pathogenesis of kidney diseases (epmc).pdf DeepDyve Pubget Overpricing