| Tissue Specificity | Heart, brain, placenta, lung, liver, muscle, kidney, retina, pancreas and fetal retinal pigment epithelium. Isoform 3 is found only in the retina. Colocalizes with RPGRIP1 in the outer segment of rod photoreceptors and cone outer segments. |
| Post Translational Modifications | Prenylated. |
| Function | Acts as a guanine-nucleotide releasing factor (GEF) for RAB8A and RAB37 by promoting the conversion of inactive RAB-GDP to the active form RAB-GTP. GEF activity towards RAB8A may facilitate ciliary trafficking by modulating ciliary intracellular localization of RAB8A. GEF activity towards RAB37 maintains autophagic homeostasis and retinal function. Involved in photoreceptor integrity. May control cilia formation by regulating actin stress filaments and cell contractility. May be involved in microtubule organization and regulation of transport in primary cilia. May play a critical role in spermatogenesis and in intraflagellar transport processes. |
| Protein Name | X-Linked Retinitis Pigmentosa Gtpase Regulator |
| Database Links | |
| Cellular Localisation | CytoplasmCytoskeletonFlagellum AxonemeGolgi ApparatusCell ProjectionCiliumIn The Retinal Photoreceptor Cell LayerLocalizes At The Connecting CiliumColocalizes With Whrn In The Photoreceptor Connecting CiliumColocalizes With Cep290 In The Photoreceptor Connecting CiliumColocalizes With Rpgrip1 In The Photoreceptor Connecting CiliumColocalizes With Rpgr At The Primary Cilia Of Epithelial CellsIsoform 6: CytoplasmMicrotubule Organizing CenterCentrosomeCilium Basal BodyCilium Axoneme |
| Alternative Antibody Names | Anti-X-Linked Retinitis Pigmentosa Gtpase Regulator antibodyAnti-RPGR antibodyAnti-RP3 antibodyAnti-XLRP3 antibody |
Information sourced from Uniprot.org
