rab escort protein-2 (REP-2, choroideraemia-like protein, CHML)

Function

• binds unprenylated Rab protein
• presents it to the catalytic Rab GGTase dimer, & remains bound to it after the geranylgeranyl transfer reaction
• component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane
• less effective than REP-1 in supporting prenylation of Rab3 family
• substitutes for REP-1 thereby preventing widespread tissue abnormalities in patients with choroideremia who lack REP-1
• interacts with Rab & Rab GGTase

Structure

• monomer
• belongs to the Rab GDI family

More general terms

• rab escort protein

Additional terms

• geranylgeranyltransferase-2 (GGTase-2, rab GGTase)

Database

• UniProt: http://www.uniprot.org/uniprot/P26374.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1122
• OMIM: https://mirror.omim.org/entry/118825