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| CDNA CLONING OF COMPONENT-A OF RAB GERANYLGERANYL TRANSFERASE AND DEMONSTRATION OF ITS ROLE AS A RAB ESCORT PROTEIN |
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| Author(s): ANDRES DA, SEABRA MC, BROWN MS, ARMSTRONG SA, SMELAND TE, CREMERS FPM, GOLDSTEIN JL |
| Source: CELL Volume: 73 Issue: 6 Pages: 1091-1099 Published: JUN 18 1993 |
| Times Cited: 213 References: 36 |
| Abstract: cDNA cloning of component A of rat Rab geranylgeranyl transferase confirms identity of the protein with the human choroideremia gene product and its resemblance to Rab3A guanine nucleotide dissociation inhibitor (GDI), which binds prenylated Rabs. In biochemical assays we demonstrate that component A binds unprenylated Rab1A, presents it to the catalytic component B, and remains bound to it after the geranylgeranyl transfer reaction. In the absence of detergents, the reaction terminates when all of component A is occupied with prenylated Rab. Detergents allow multiple rounds of catalysis, apparently by dissociating the component A-Rab complex and thus allowing recycling of component A. Within the cell, component A may be regenerated by transferring its prenylated Rab to a protein acceptor, such as Rab3A GDI. In view of its function in escorting Rab proteins during and presumably after the prenyl transfer reaction, we propose to rename component A as Rab escort protein (REP). A genetic defect in REP underlies human choroideremia, a disease of retinal degeneration. |
| Document Type: Article |
| Language: English |
| Reprint Address: ANDRES, DA (reprint author), UNIV TEXAS, SW MED CTR, DEPT MOLEC GENET, DALLAS, TX 75235 USA |
| Publisher: CELL PRESS, 1050 MASSACHUSETTES AVE, CIRCULATION DEPT, CAMBRIDGE, MA 02138 |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology |
| IDS Number: LH548 |
| ISSN: 0092-8674 |
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