InterPro : IPR001738

Name  Rab escort (choroideraemia) protein Short Name  Rab_escort
Type  Family Description  Rab proteins constitute a family of small GTPases that serve a regulatory role in vesicular membrane traffic [, ]; C-terminal geranylgeranylation is crucial for their membrane association and function. This post-translational modification is catalysed by Rab geranylgeranyl transferase (Rab-GGTase), a multi-subunit enzyme that contains a catalytic heterodimer and an accessory component, termed Rab escort protein (REP)-1 [, ]. REP-1 presents newly-synthesised Rab proteins to the catalytic component, and forms a stable complex with the prenylated proteins following the transfer reaction.cDNA cloning of component A of rat Rab geranylgeranyl transferase (REP) confirms its resemblance to Rab3A guanine nucleotide dissociation inhibitor (GDI) and its identity with the human choroideremia gene product []. A genetic defect in REP underlies human choroideremia. Choroideraemia (or tapetochoroidal dystrophy) is a common form of X-linked blindness characterised by progressive dystrophy of the choroid, retinal pigment epithelium and retina [, , ].
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InterPro protein domain ID --> Contigs


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0 Child Features

0 Contains

0 Found In

1 Parent Features

Id Name Short Name Type
IPR016664 Rab protein geranylgeranyltransferase component A, eukaryota Rab_geranylTrfase_A_euk Family

5 Publications

First Author Title Year Journal Volume Pages
Alexandrov K Rab escort protein-1 is a multifunctional protein that accompanies newly prenylated rab proteins to their target membranes. 1994 EMBO J 13 5262-73
van Bokhoven H Cloning and characterization of the human choroideremia gene. 1994 Hum Mol Genet 3 1041-6
Andres DA cDNA cloning of component A of Rab geranylgeranyl transferase and demonstration of its role as a Rab escort protein. 1993 Cell 73 1091-9
Cremers FP Cloning of a gene that is rearranged in patients with choroideraemia. 1990 Nature 347 674-7
Merry DE Isolation of a candidate gene for choroideremia. 1992 Proc Natl Acad Sci U S A 89 2135-9

To cite PlanMine, please refer to the following publication:

Brandl, H., Moon, H., Vila-Farré, M., Liu, S.-Y., Henry, I., & Rink, J. C.
PlanMine - a mineable resource of planarian biology and biodiversity.
Nucleic Acids Research, gkv1148. doi:10.1093/nar/gkv1148 (2015)