rab escort protein-1 (REP-1, TCD protein, p95 choroideraemia gene product, p95CHM, CHM, REP1, TCD)
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Function
- rab escort protein, removing prenylated rab3A from the catalytic subunits of geranylgeranyl transferase-2 so that further proteins can be modified
- binds unprenylated Rab proteins, 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
- interacts with Rab & Rab GGTase
Structure
Pathology
- defects in CHM are the cause of choroideremia
More general terms
Additional terms
References
- ↑ Boguski MS & McCormick F Proteins regulating Ras and its relative. Nature 366:643 1993 PMID: https://www.ncbi.nlm.nih.gov/pubmed/8259209
- ↑ Casey PJ Protein lipidation in cell signaling. Science 268:221 1995 PMID: https://www.ncbi.nlm.nih.gov/pubmed/7716512
- ↑ Mutations of the REP1 gene Retina International's Scientific newsletter http://www.retina-international.com/sci-news/repmut.htm
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=CHM
- ↑ UniProt http://www.uniprot.org/uniprot/P24386.html