Skip to main content
Article
In and Out of the ER: Protein Folding, Quality Control, Degradation, and Related Human Diseases
Physiological Review (2007)
  • Daniel Hebert, University of Massachusetts - Amherst
  • M. Molinari
Abstract

A substantial fraction of eukaryotic gene products are synthesized by ribosomes attached at the cytosolic face of the endoplasmic reticulum (ER) membrane. These polypeptides enter cotranslationally in the ER lumen, which contains resident molecular chaperones and folding factors that assist their maturation. Native proteins are released from the ER lumen and are transported through the secretory pathway to their final intra- or extracellular destination. Folding-defective polypeptides are exported across the ER membrane into the cytosol and destroyed. Cellular and organismal homeostasis relies on a balanced activity of the ER folding, quality control, and degradation machineries as shown by the dozens of human diseases related to defective maturation or disposal of individual polypeptides generated in the ER.

Publication Date
October, 2007
Publisher Statement
Doi: 10.​1152/​physrev.​00050.​2006
Citation Information
Daniel Hebert and M. Molinari. "In and Out of the ER: Protein Folding, Quality Control, Degradation, and Related Human Diseases" Physiological Review Vol. 87 Iss. 4 (2007)
Available at: http://works.bepress.com/daniel_hebert/11/