Marfan Syndrome Mutations Predominantly Alter Fibrillin Domain Folding

Abstract

The RGK family of small G-proteins, including Rad, Gem, Rem1 and Rem2, are inducibly expressed in various mammalian tissues and interact with voltage-dependent calcium channels and Rho kinase. To test whether RGK proteins undergo a nucleotide-induced conformational change, we determined the crystallographic structures of G-domains from Rad and Rem2 bound to GppNHp, a GTP analog, and compared them to their respective GDP bound structures. Also, we characterized the nucleotide-binding properties and conformations for Gem, Rad, and several structure-based mutants using fluores