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

Journal article
Authors Kara Schneider
Thomas Nyström
Per O Widlund
Published in Frontiers in Molecular Neuroscience
Volume 11
ISSN 1662-5099
Publication year 2018
Published at Institute of Biomedicine, Department of Microbiology and Immunology
Language en
Keywords protein quality control, spatial protein quality control, protein misfolding, misfolding model, green fluorescent protein, inclusion-body formation, alpha-synuclein, saccharomyces-cerevisiae, asymmetric inheritance, damaged proteins, cytosolic proteins, intranuclear inclusions, polyglutamine toxicity, endoplasmic-reticulum, Neurosciences & Neurology, ates of america, v111, p8049, otocols, v832, p489, ates of america, v105, p6439, ates of america, v103, p11045, ates of america, v105, p7732, hroder h, 1993, embo journal, v12, p4137
Subject categories Neurology


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. Saccharomyces cerevisiae has been used extensively to elucidate PQC pathways and general evolutionary conservation of the PQC machinery has led to the development of several useful S. cerevisiae 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 S. cerevisiae, as well as the misfolding proteins that have been shown to be subject to spatial quality control in S. cerevisiae models of human neurological diseases.

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