Conformational Stability and Pathogenic Misfolding of the Integral Membrane Protein PMP22

Conformational Stability and Pathogenic Misfolding of the Integral Membrane Protein PMP22
Schlebach et al. 2015 (Sanders Lab - Vanderbilt) - JACS

• Understanding of reactions that limit assembly and trafficking of membrane proteins remains superficial
• Assess relationship between stability of an MP and degree to which its retained by the quality control system
• Looked at 12 variants of PMP22 - linked to charcot-marie-tooth disease (CMT)
• Influence ZnII mediated folding proportional to reduction in trafficking efficiency
• Mutations important for energetics of PMP22 folding and a molecular basis for CMT