InterPro domain: IPR039093

XRP2, also known as RP2, acts as a GTPase-activating protein (GAP). It has been shown to bind the GTP form of the small G protein Arf-like 3 (Arl3) [ 1 ]; this interaction regulates the trafficking of ciliary tip kinesins [ 2 ]. Mutations in the retinitis pigmentosa 2 (RP2) gene cause a severe form of X-linked retinal degeneration [ 3 ].

1. The retinitis pigmentosa 2 gene product is a GTPase-activating protein for Arf-like 3. Nat. Struct. Mol. Biol. 15, 373-80
2. Arl3 and RP2 regulate the trafficking of ciliary tip kinesins. Hum. Mol. Genet. 26, 2480-2492
3. Localization in the human retina of the X-linked retinitis pigmentosa protein RP2, its homologue cofactor C and the RP2 interacting protein Arl3. Hum. Mol. Genet. 11, 3065-74