Rab escort protein 1 (REP1) in intracellular traffic: a functional and pathophysiological overview.
In: Ophthalmic Genetics, Jg. 25 (2004-06-01), Heft 2, S. 101-110
Online
academicJournal
Zugriff:
The intracellular distribution of proteins, compartments, substrates, and products is an active process called intracellular traffic. Control of intracellular traffic is established by small GTP-binding proteins (Rab proteins). Rab proteins are modified by geranyl-geranyl moieties necessary for membrane association and target-protein recognition. Geranyl-geranyl groups are transferred to Rab proteins by geranyl-geranyl transferase 2 (GGTase2). GGTase2 requires Rab escort protein 1 (REP1) to bind Rab proteins. REP1 null mutations underlie an X-linked retinal degeneration called choroideremia (CHM). This review summarizes the current biochemical and clinical knowledge on REP1 and CHM. [ABSTRACT FROM AUTHOR]
Titel: |
Rab escort protein 1 (REP1) in intracellular traffic: a functional and pathophysiological overview.
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Autor/in / Beteiligte Person: | Preising, Markus N. ; Ayuso, Carmen |
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Zeitschrift: | Ophthalmic Genetics, Jg. 25 (2004-06-01), Heft 2, S. 101-110 |
Veröffentlichung: | 2004 |
Medientyp: | academicJournal |
ISSN: | 1381-6810 (print) |
DOI: | 10.1080/13816810490514333 |
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