Use my Search Websuite to scan PubMed, PMCentral, Journal Hosts and Journal Archives, FullText. Kick-your-searchterm to multiple Engines kick-your-query now !>

A dictionary by aggregated review articles of nephrology, medicine and the life sciences Your one-stop-run pathway from word to the immediate pdf of peer-reviewed on-topic knowledge.

10.1016/j.preteyeres.2017.01.005 http://scihub22266oqcxt.onion/10.1016/j.preteyeres.2017.01.005 C5441932!5441932 !28111324     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=28111324 &cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215 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 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\28111324 .jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       Prog+Retin+Eye+Res 2017 ; 58 (ä): 70-88 Nephropedia Template TP {{tp|p=28111324 |t=2017. Bestrophinopathy: An RPE-photoreceptor interface disease |pdf=|usr=}}{{28111324 }} gab.com Text Bestrophinopathy: An RPE-photoreceptor interface disease JO: Prog Retin Eye Res http://www.kidney.de/pget.php?&tw=1&pmid=28111324 #FOAvip Bestrophinopathies, one of the most common forms of inherited macular degenerations, are caused by mutations in the BEST1 gene expressed in the retinal pigment epithelium (RPE). Both human and canine BEST1-linked maculopathies are characterized by abnormal accumulation of autofluorescent material within RPE cells and bilateral macular or multifocal lesions; however, the specific mechanism leading to the formation of these lesions remains unclear. We now provide an overview of the current state of knowledge on the molecular pathology of bestrophinopathies, and explore factors promoting formation of RPE-neuroretinal separations, using the first spontaneous animal model of BEST1-associated retinopathies, canine Best (cBest). Here, we characterize the nature of the autofluorescent RPE cell inclusions and report matching spectral signatures of RPE-associated fluorophores between human and canine retinae, indicating an analogous composition of endogenous RPE deposits in Best Vitelliform Macular Dystrophy (BVMD) patients and its canine disease model. This study also exposes a range of biochemical and structural abnormalities at the RPE-photoreceptor interface related to the impaired cone-associated microvillar ensheathment and compromised insoluble interphotoreceptor matrix (IPM), the major pathological culprits responsible for weakening of the RPE-neuroretina interactions, and consequently, formation of vitelliform lesions. These salient alterations detected at the RPE apical domain in cBest as well as in BVMD- and ARB-hiPSC-RPE model systems provide novel insights into the pathological mechanism of BEST1-linked disorders that will allow for development of critical outcome measures guiding therapeutic strategies for bestrophinopathies. Twit Text FOAVip Bestrophinopathy: An RPE-photoreceptor interface disease ????Prog Retin Eye Res http://www.kidney.de/pget.php?&tw=1&pmid=28111324 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=28111324 #FOAvip English Wikipedia <ref>{{Cite pmid|28111324 }}</ref> Bestrophinopathy: An RPE-photoreceptor interface disease #MMPMID28111324 Guziewicz KE ; Sinha D ; Gómez NM ; Zorych K ; Dutrow EV ; Dhingra A ; Mullins RF ; Stone EM ; Gamm DM ; Boesze-Battaglia K ; Aguirre GD Prog Retin Eye Res 2017[May]; 58 (ä): 70-88 PMID28111324 show ga Bestrophinopathies, one of the most common forms of inherited macular degenerations, are caused by mutations in the BEST1 gene expressed in the retinal pigment epithelium (RPE). Both human and canine BEST1-linked maculopathies are characterized by abnormal accumulation of autofluorescent material within RPE cells and bilateral macular or multifocal lesions; however, the specific mechanism leading to the formation of these lesions remains unclear. We now provide an overview of the current state of knowledge on the molecular pathology of bestrophinopathies, and explore factors promoting formation of RPE-neuroretinal separations, using the first spontaneous animal model of BEST1-associated retinopathies, canine Best (cBest). Here, we characterize the nature of the autofluorescent RPE cell inclusions and report matching spectral signatures of RPE-associated fluorophores between human and canine retinae, indicating an analogous composition of endogenous RPE deposits in Best Vitelliform Macular Dystrophy (BVMD) patients and its canine disease model. This study also exposes a range of biochemical and structural abnormalities at the RPE-photoreceptor interface related to the impaired cone-associated microvillar ensheathment and compromised insoluble interphotoreceptor matrix (IPM), the major pathological culprits responsible for weakening of the RPE-neuroretina interactions, and consequently, formation of vitelliform lesions. These salient alterations detected at the RPE apical domain in cBest as well as in BVMD- and ARB-hiPSC-RPE model systems provide novel insights into the pathological mechanism of BEST1-linked disorders that will allow for development of critical outcome measures guiding therapeutic strategies for bestrophinopathies. |*Eye Diseases, Hereditary/genetics/metabolism/therapy [MESH] |*Gene Expression Regulation [MESH] |*Retinal Diseases/genetics/metabolism/therapy [MESH] |Animals [MESH] |Bestrophins/biosynthesis/*genetics [MESH] |DNA/*genetics [MESH] |Genetic Therapy/*methods [MESH] |Humans [MESH] |Retinal Cone Photoreceptor Cells/metabolism/*pathology [MESH]Bestrophinopathy: An RPE-photoreceptor interface disease (epmc).pdf DeepDyve Pubget Overpricing