Article ID Journal Published Year Pages File Type
4033865 Vision Research 2012 7 Pages PDF
Abstract

Expressed ubiquitously, PrBP/δ functions as chaperone/co-factor in the transport of a subset of prenylated proteins. PrBP/δ features an immunoglobulin-like β-sandwich fold for lipid binding, and interacts with diverse partners. PrBP/δ binds both C-terminal C15 and C20 prenyl side chains of phototransduction polypeptides and small GTP-binding (G) proteins of the Ras superfamily. PrBP/δ also interacts with the small GTPases, ARL2 and ARL3, which act as release factors (GDFs) for prenylated cargo. Targeted deletion of the mouse Pde6d gene encoding PrBP/δ resulted in impeded trafficking to the outer segments of GRK1 and cone PDE6 which are predicted to be farnesylated and geranylgeranylated, respectively. Rod and cone transducin trafficking was largely unaffected. These trafficking defects produce progressive cone-rod dystrophy in the Pde6d−/− mouse.

► Ubiquitously-expressed PrBP/δ belongs to the UNC119 gene family. ► PrBP/δ forms an immunoglobulin-like β-sandwich fold into which prenyl chains may insert. ► PrBP/δ interacts with a subset of prenylated G proteins of the ras gene family. ► PrBP/δ controls trafficking of GRK1 and PDE6 to photoreceptor outer segments. ► PrBP/δ deletion in mouse causes recessive cone/rod dystrophy.

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Life Sciences Neuroscience Sensory Systems
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