Prenylation of Rab GTPases: molecular mechanisms and involvement in genetic disease

被引：137

作者：

Pereira-Leal, JB ^{[1
]}

Hume, AN ^{[1
]}

Seabra, MC ^{[1
]}

机构：

[1] Univ London Imperial Coll Sci Technol & Med, Div Biomed Sci, Cell & Mol Biol Sect, London SW7 2AZ, England

来源：

FEBS LETTERS | 2001年 / 498卷 / 2-3期

关键词：

Rab; GTP-binding protein; protein prenylation; protein traffic; choroideremia; Hermansky-Pudlak syndrome; Griscelli disease;

D O I：

10.1016/S0014-5793(01)02483-8

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Small GTPases of the Rab family regulate membrane transport pathways. More than 50 mammalian Rab proteins are known, many with transport step-specific localisation. Rabs must associate with cellular membranes for activity and membrane attachment is mediated by prenyl (geranylgeranyl) post-translational modification. Mutations in genes encoding proteins essential for the geranylgeranylation reaction, Rab escort protein and Rab geranylgeranyl transferase, underlie genetic diseases. Choroideremia patients have loss of function mutations in REP1 and the murine Hermansky-Pudlak syndrome model gunmetal possesses a splice-site mutation in the a-subunit of RGGT. Here we discuss recent insights into Rab prenylation and advances towards our understanding of both diseases. (C) 2001 Federation of European Biochemical Societies. Published by Elsevier Science B,V, All rights reserved.

引用

页码：197 / 200

页数：4

共 46 条

[1] RAB ESCORT PROTEIN-1 IS A MULTIFUNCTIONAL PROTEIN THAT ACCOMPANIES NEWLY PRENYLATED RAB PROTEINS TO THEIR TARGET MEMBRANES [J].