- Christianson, John C;
- Olzmann, James A;
- Shaler, Thomas A;
- Sowa, Mathew E;
- Bennett, Eric J;
- Richter, Caleb M;
- Tyler, Ryan E;
- Greenblatt, Ethan J;
- Wade Harper, J;
- Kopito, Ron R
Proteins that fail to correctly fold or assemble into oligomeric complexes in the endoplasmic reticulum (ER) are degraded by a ubiquitin- and proteasome-dependent process known as ER-associated degradation (ERAD). Although many individual components of the ERAD system have been identified, how these proteins are organized into a functional network that coordinates recognition, ubiquitylation and dislocation of substrates across the ER membrane is not well understood. We have investigated the functional organization of the mammalian ERAD system using a systems-level strategy that integrates proteomics, functional genomics and the transcriptional response to ER stress. This analysis supports an adaptive organization for the mammalian ERAD machinery and reveals a number of metazoan-specific genes not previously linked to ERAD.