1vcb

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spinqualitylabelsall models 1vcb, resolution 2.7Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

THE VHL-ELONGINC-ELONGINB STRUCTURE

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

Overview

Mutation of the VHL tumor suppressor is associated with the inherited von, Hippel-Lindau (VHL) cancer syndrome and the majority of kidney cancers., VHL binds the ElonginC-ElonginB complex and regulates levels of, hypoxia-inducible proteins. The structure of the ternary complex at 2.7, angstrom resolution shows two interfaces, one between VHL and ElonginC and, another between ElonginC and ElonginB. Tumorigenic mutations frequently, occur in a 35-residue domain of VHL responsible for ElonginC binding. A, mutational patch on a separate domain of VHL indicates a second, macromolecular binding site. The structure extends the similarities to the, SCF (Skp1-Cul1-F-box protein) complex that targets proteins for, degradation, supporting the hypothesis that VHL may function in an, analogous pathway.

Disease

Known diseases associated with this structure: Hemangioblastoma, cerebellar, somatic OMIM:[608537], Pheochromocytoma OMIM:[608537], Polycythemia, benign familial OMIM:[608537], Renal cell carcinoma, somatic OMIM:[608537], von Hippel-Lindau syndrome OMIM:[608537]

About this Structure

1VCB is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structure of the VHL-ElonginC-ElonginB complex: implications for VHL tumor suppressor function., Stebbins CE, Kaelin WG Jr, Pavletich NP, Science. 1999 Apr 16;284(5413):455-61. PMID:10205047

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