[HTML][HTML] Deficient Geranylgeranylation of Ram/Rab27 in Choroideremia (∗)

MC Seabra, YK Ho, JS Anant - Journal of Biological Chemistry, 1995 - ASBMB
Choroideremia, an X-linked form of retinal degeneration, results from defects in the Rab
escort protein-1 (REP-1) gene. REP-1 and REP-2 assist in the attachment of geranylgeranyl
groups to Rab GTPases, a modification essential for their action as molecular switches
regulating intracellular vesicular transport. If Rabs that depend preferentially on REP-1 for
prenylation exist, they will accumulate unprenylated in choroideremia cells. Using
recombinant Rab geranylgeranyl transferase and REPs to label unprenylated cytosolic …