Studying Spatial Protein Quality Control, Proteopathies, and Aging Using Different Model Misfolding Proteins in S. cerevisiae

• Published in: Frontiers in molecular neuroscience Vol. 11; p. 249
• Main Authors: Schneider, Kara L., Nyström, Thomas, Widlund, Per O.
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers Research Foundation 23.07.2018; Frontiers Media S.A
• Subjects: 1993; Age; Aging; alpha-synuclein; asymmetric inheritance; ates of america; Cell division; cytosolic proteins; damaged proteins; Disease; embo journal; endoplasmic-reticulum; Evolutionary conservation; green fluorescent protein; hroder h; inclusion-body formation; inclusions; intranuclear inclusions; misfolding model; Neurologi; Neurological diseases; Neurology; Neuroscience; Neurosciences & Neurology; otocols; p11045; p4137; p489; p6439; p7732; p8049; polyglutamine toxicity; Protein folding; protein misfolding; protein quality control; Proteins; Proteomes; Quality control; Saccharomyces cerevisiae; spatial protein quality control; Stem cells; Toxicity; v103; v105; v111; v12; v832; misfolding model; protein misfolding; cell stress; protein quality control; aging; temperature-sensitive; inclusions; spatial protein quality control
• ISSN: 1662-5099; 1662-5099
• DOI: 10.3389/fnmol.2018.00249

Protein quality control (PQC) is critical to maintain a functioning proteome. Misfolded or toxic proteins are either refolded or degraded by a system of temporal quality control and can also be sequestered into aggregates or inclusions by a system of spatial quality control. Breakdown of this concerted PQC network with age leads to an increased risk for the onset of disease, particularly neurological disease. has been used extensively to elucidate PQC pathways and general evolutionary conservation of the PQC machinery has led to the development of several useful models of human neurological diseases. Key to both of these types of studies has been the development of several different model misfolding proteins, which are used to challenge and monitor the PQC machinery. In this review, we summarize and compare the model misfolding proteins that have been used to specifically study spatial PQC in , as well as the misfolding proteins that have been shown to be subject to spatial quality control in models of human neurological diseases.