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  4. Retina-specific loss of Ikbkap/Elp1 causes mitochondrial dysfunction that leads to selective retinal ganglion cell degeneration in a mouse model of familial dysautonomia

Retina-specific loss of Ikbkap/Elp1 causes mitochondrial dysfunction that leads to selective retinal ganglion cell degeneration in a mouse model of familial dysautonomia

Ueki Y, Shchepetkina V, Lefcort F (2018) Retina-specific loss of Ikbkap/Elp1 causes mitochondrial dysfunction that leads to selective retinal ganglion cell degeneration in a mouse model of familial dysautonomia. Dis Model Mech 11(7):dmm033746. doi: 10.1242/dmm.033746 PMID: 29929962

Objective: To determine the pathophysiological mechanisms that are triggered by the absence of IKAP (inhibitor of kappa B kinase complex-associated protein) in the retina.

Summary: The loss of IKAP caused progressive degeneration of retinal ganglion cells (RGCs) by 1 month of age. There was no loss of melanopsin+ intrinsically photosensitive RGCs at 18 months. RGCs were the only cell type that degenerated, with the survival of other retinal neurons unaffected.

Usage: Immunohistochemistry (IHC), RGC Counts, and H&E staining – Anti-melanopsin (1:5000).

Related Products: Melanopsin Rabbit Polyclonal (Cat. #AB-N38)

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