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Overview
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REP1 is the component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Mutations in this protein are a cause of a type of retinal degeneration, called choroideremia.
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