Rab Escort Protein 1 (REP1)

Rab Escort Protein 1 (REP1) is a chaperone protein essential for the prenylation and membrane targeting of Rab GTPases. It binds newly synthesized, unprenylated Rab GTPases and presents them to Rab geranylgeranyl transferase (RabGGTase) for modification. This lipid modification is crucial for proper membrane localization and function of Rab GTPases, which regulate intracellular vesicular trafficking. Mutations in the gene encoding REP1, CHM, cause choroideremia, an X-linked retinal degenerative disease, highlighting REP1's importance in ocular tissue function.

Other names

Rab proteins geranylgeranyltransferase component A 1

Not applicable - REP1 is not directly targeted by drugs but is a critical protein for the function of Rab GTPases, which could be indirectly affected.