RGD Reference Report - Structure of the Rab7:REP-1 complex: insights into the mechanism of Rab prenylation and choroideremia disease. - Rat Genome Database

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Structure of the Rab7:REP-1 complex: insights into the mechanism of Rab prenylation and choroideremia disease.

Authors: Rak, A  Pylypenko, O  Niculae, A  Pyatkov, K  Goody, RS  Alexandrov, K 
Citation: Rak A, etal., Cell. 2004 Jun 11;117(6):749-60.
RGD ID: 8553294
Pubmed: PMID:15186776   (View Abstract at PubMed)
DOI: DOI:10.1016/j.cell.2004.05.017   (Journal Full-text)

Members of the RabGDI/REP family serve as multifunctional regulators of the Rab family of GTP binding proteins. Mutations in members of this family, such as REP-1, lead to abnormalities, including progressive retinal degradation (choroideremia) in humans. The crystal structures of the REP-1 protein in complex with monoprenylated or C-terminally truncated Rab7 proteins revealed that Rab7 interacts with the Rab binding platform of REP-1 via an extended interface involving the Switch 1 and 2 regions. The C terminus of the REP-1 molecule functions as a mobile lid covering a conserved hydrophobic patch on the surface of REP-1 that in the complex coordinates the C terminus of Rab proteins. Using semisynthetic fluorescent Rab27A, we demonstrate that although Rab27A can be prenylated by REP-2, this reaction can be effectively inhibited by other Rab proteins, providing a possible explanation for the accumulation of unprenylated Rab27A in choroideremia.

Gene Ontology Annotations    Click to see Annotation Detail View

Cellular Component
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Rab-protein geranylgeranyltransferase complex part_ofIDA 8553294PMID:15186776UniProt 

Molecular Function
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
protein binding enablesIPIUniProtKB:P095278553294PMID:15186776IntAct 
protein binding enablesIPIUniProtKB:P377278553294PMID:15186776IntAct 
small GTPase binding enablesIDA 8553294PMID:15186776UniProt 

Objects Annotated

Genes (Rattus norvegicus)
Chm  (CHM Rab escort protein)
Rab7a  (RAB7A, member RAS oncogene family)


Additional Information