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10.1167/iovs.13-13536 http://scihub22266oqcxt.onion/10.1167/iovs.13-13536 C4587794!4587794 !24346621     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=24346621 &cmd=llinks): Failed to open stream: HTTP request failed! 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A perspective on the role of the extracellular matrix in progressive retinal degenerative disorders |pdf=|usr=}}{{24346621 }} gab.com Text A perspective on the role of the extracellular matrix in progressive retinal degenerative disorders JO: Invest Ophthalmol Vis Sci http://www.kidney.de/pget.php?&tw=1&pmid=24346621 #FOAvip Progressive inherited retinal degenerative disorders (PIRDDs) are the leading cause of blindness in developed countries, with AMD and RP constituting the majority of PIRDDs. Currently, over 8 million Americans have PIRDDs, and that number is estimated to drastically increase by the end of this decade. Although a mutant protein is expressed starting early during retinal development in patients with PIRDDs, symptoms of retinal degeneration do not manifest until much later. Historically, research has focused on understanding the role a mutation has in the function of a protein and what role the mutant protein has in the disease process. However, it remains unknown why the disease, irrespective of the mutation, manifests clinically much later in life, while cellular indicators of disease (e.g., accumulation of toxic protein products and cell death) occur throughout early and middle life. Herein, we propose that there exists a time point at which the degenerative process is accelerated, leading to the appearance of clinical symptoms. This point is defined by structural disruptions of the extracellular matrix (ECM). Death of a critical number of ECM-maintaining mutant protein-expressing retinal cells contributes to that break point in the degenerative process. Therefore, it is important to understand the changes occurring at the ECM during PIRDDs and to take that into account when therapeutic approaches are designed. Twit Text FOAVip A perspective on the role of the extracellular matrix in progressive retinal degenerative disorders ????Invest Ophthalmol Vis Sci http://www.kidney.de/pget.php?&tw=1&pmid=24346621 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24346621 #FOAvip English Wikipedia <ref>{{Cite pmid|24346621 }}</ref> A perspective on the role of the extracellular matrix in progressive retinal degenerative disorders #MMPMID24346621 Al-Ubaidi MR ; Naash MI ; Conley SM Invest Ophthalmol Vis Sci 2013[Dec]; 54 (13 ): 8119-24 PMID24346621 show ga Progressive inherited retinal degenerative disorders (PIRDDs) are the leading cause of blindness in developed countries, with AMD and RP constituting the majority of PIRDDs. Currently, over 8 million Americans have PIRDDs, and that number is estimated to drastically increase by the end of this decade. Although a mutant protein is expressed starting early during retinal development in patients with PIRDDs, symptoms of retinal degeneration do not manifest until much later. Historically, research has focused on understanding the role a mutation has in the function of a protein and what role the mutant protein has in the disease process. However, it remains unknown why the disease, irrespective of the mutation, manifests clinically much later in life, while cellular indicators of disease (e.g., accumulation of toxic protein products and cell death) occur throughout early and middle life. Herein, we propose that there exists a time point at which the degenerative process is accelerated, leading to the appearance of clinical symptoms. This point is defined by structural disruptions of the extracellular matrix (ECM). Death of a critical number of ECM-maintaining mutant protein-expressing retinal cells contributes to that break point in the degenerative process. Therefore, it is important to understand the changes occurring at the ECM during PIRDDs and to take that into account when therapeutic approaches are designed. |*Aging [MESH] |*Retinal Degeneration/epidemiology/genetics/metabolism [MESH] |Animals [MESH] |Disease Progression [MESH] |Extracellular Matrix/*physiology [MESH] |Genetic Predisposition to Disease [MESH] |Global Health [MESH] |Humans [MESH]A perspective on the role of the extracellular matrix in progressive r(epmc).pdf DeepDyve Pubget Overpricing