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GARP2 accelerates retinal degeneration in rod cGMP-gated cation channel β-subunit knockout mice.

Sci Rep. 2017; 
DeRamusMarci L,StacksDelores A,ZhangYouwen,HuisinghCarrie E,McGwinGerald,PittlerStev
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Proteins, Expression, Isolation and Analysis Immunohistochemistry was performed as previously described using a custom designed purified antibody (Genscript) with the epitope (N-Cngb1; QEPPEPKDPPKPPGC) (1:50)14. Get A Quote

摘要

The Cngb1 locus-encoded β-subunit of rod cGMP-gated cation channel and associated glutamic acid rich proteins (GARPs) are required for phototransduction, disk morphogenesis, and rod structural integrity. To probe individual protein structure/function of the GARPs, we have characterized several transgenic mouse lines selectively restoring GARPs on a Cngb1 knockout (X1) mouse background. Optical coherence tomography (OCT), light and transmission electron microscopy (TEM), and electroretinography (ERG) were used to analyze 6 genotypes including WT at three and ten weeks postnatal. Comparison of aligned histology/OCT images demonstrated that GARP2 accelerates the rate of degeneration. ERG results are con... More

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