title: Protein quality control: the who's who, the where's and therapeutic escapes.
abstract: In cells the quality of newly synthesized proteins is monitored in regard to proper folding and correct assembly in the early secretory pathway, the cytosol and the nucleoplasm. Proteins recognized as non-native in the ER will be removed and degraded by a process termed ERAD. ERAD of aberrant proteins is accompanied by various changes of cellular organelles and results in protein folding diseases. This review focuses on how the immunocytochemical labeling and electron microscopic analyses have helped to disclose the in situ subcellular distribution pattern of some of the key machinery proteins of the cellular protein quality control, the organelle changes due to the presence of misfolded proteins, and the efficiency of synthetic chaperones to rescue disease-causing trafficking defects of aberrant proteins.
authors: Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, 8091 Zurich, Switzerland. jurgen.roth@bluewin.ch
authors: Jürgen
authors: J
authors: Roth
authors: Gary Hin-Fai
authors: GH
authors: Yam
authors: Jingyu
authors: J
authors: Fan
authors: Kiyoko
authors: K
authors: Hirano
authors: Katarina
authors: K
authors: Gaplovska-Kysela
authors: Valerie
authors: V
authors: Le Fourn
authors: Bruno
authors: B
authors: Guhl
authors: Roger
authors: R
authors: Santimaria
authors: Tania
authors: T
authors: Torossi
authors: Martin
authors: M
authors: Ziak
authors: Christian
authors: C
authors: Zuber
mesh_terms: D015195:Drug Design; D004721:Endoplasmic Reticulum / Q000378:metabolism* / Q000648:ultrastructure; D006801:Humans; D008565:Membrane Proteins / Q000502:physiology; D008661:Metabolism, Inborn Errors / Q000188:drug therapy / Q000209:etiology; D018832:Molecular Chaperones / Q000378:metabolism* / Q000627:therapeutic use; D017510:Protein Folding; D011506:Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: C486654:EDEM1 protein, human; D008565:Membrane Proteins; D018832:Molecular Chaperones; D011506:Proteins