Flanking Regions Determine the Structure of the Poly-Glutamine in Huntingtin through Mechanisms Common among Glutamine-Rich Human Proteins

Abstract

Huntingtin exon1 (httex1) is the causative agent of Huntington's disease, a deadly neurodegenerative disorder. Combining site-specific labeling with NMR and computational modeling, Urbanek et al. have characterized the features governing the structure of a non-pathogenic version of httex1. The found structural traits are also present in many human glutamine-rich proteins.