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

Sci Rep. 2017 Feb 15;7:42545
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摘要


The Cngb1 locus-encoded β-subunit of rod cGMP-gated cation channel and associated glutamic acid rich proteins are required for phototransduction, disk morphogenesis, and rod structural integrity. To probe individual protein structure/function of the we have characterized several transgenic mouse lines selectively restoring 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 accelerates the rate of degeneration. ERG results are consistent with the structural analyses showing the greatest attenuation of function when Gduanyu372 is present. Even 100-fold or more overexpression of could not accelerate degeneration as rapidly as and when co-expressed Gduanyu371 attenuated the structural and functional deficits elicited by These results indicate that the Gduanyu37s are not fully interchangeable and thus, likely have separate and distinct functions in the photoreceptor. We also present a uniform murine OCT layer naming nomenclature system that is consistent with human retina layer designations to standardize murine OCT, which will facilitate data evaluation across different laboratories.

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