Rab escort protein

Rab escort protein 1 also known as rab proteins geranylgeranyltransferase component A 1 is an is_associated_with::enzyme that in humans is encoded by the CHM is_associated_with::gene.

Function
This gene encodes 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. Rab GTPases need to be geranylgeranyled on either one or two is_associated_with::cysteine residues in their is_associated_with::C-terminus to localize to the correct intracellular membrane.

Interactions
CHM (gene) has been shown to interact with is_associated_with::RAB1A, is_associated_with::RAB7A and is_associated_with::RAB3A.

Clinical significance
Mutations in this gene are a cause of is_associated_with::choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina.