Domain

von Hippel-Lindau disease tumour suppressor, beta/alpha domain (IPR022772)

Short name: VHL_tumour_suppress_b/a_dom

Domain relationships

None.

Description

Von Hippel-Lindau disease tumor suppressor (VHL) has two domains: a roughly 100-residue N-terminal domain rich in beta sheet (beta domain) and a smaller alpha-helical domain (alpha domain), held together by two linkers and a polar interface. A large portion of the alpha domain surface, and a small portion of the beta domain, interact with ElonginC. About half of the tumorigenic mutations map to the alpha domain and its residues that contact ElonginC. The remaining mutations map to the beta domain, and significantly, to a beta domain surface patch uninvolved in ElonginC binding. This suggests that two intact macromolecular binding sites may be required for the tumor suppressor effects of VHL [PMID: 10205047].

This entry represents both beta and alpha domains of VHL.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
SUPERFAMILY
Pfam