Neuron 2017 Jul;95(1):33-50
École Normale Supérieure, Institut de Biologie de l'ENS (IBENS), INSERM, CNRS, PSL Research University, 46 Rue d'Ulm, 75005 Paris, France. Electronic address:
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Exp Mol Med 2015 Mar 13;47:e147. Epub 2015 Mar 13.
Protein Metabolism Medical Research Center and Department of Biomedical Sciences, College of Medicine, Seoul National University, Seoul, Korea.
Mammalian cells remove misfolded proteins using various proteolytic systems, including the ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and macroautophagy. The majority of misfolded proteins are degraded by the UPS, in which Ub-conjugated substrates are deubiquitinated, unfolded and cleaved into small peptides when passing through the narrow chamber of the proteasome. The substrates that expose a specific degradation signal, the KFERQ sequence motif, can be delivered to and degraded in lysosomes via the CMA. Read More
J Biol Chem 2014 Jul 23;289(29):19862-8. Epub 2014 May 23.
From the Medical Research Council (MRC) Toxicology Unit, Hodgkin Building, University of Leicester, Lancaster Road, Leicester LE1 9HN, United Kingdom
Neurodegenerative diseases are characterized by the aggregation of misfolded proteins in the brain. Among these disorders are the prion diseases, which are transmissible, and in which the misfolded proteins ("prions") are also the infectious agent. Increasingly, it appears that misfolded proteins in Alzheimer and Parkinson diseases and the tauopathies also propagate in a "prion-like" manner. Read More
Int J Mol Sci 2009 Jun 3;10(6):2510-57. Epub 2009 Jun 3.
University of the Witwatersrand, Department of Pharmacy and Pharmacology, Parktown, Johannesburg, South Africa.
The term neurodegenerative disorders, encompasses a variety of underlying conditions, sporadic and/or familial and are characterized by the persistent loss of neuronal subtypes. These disorders can disrupt molecular pathways, synapses, neuronal subpopulations and local circuits in specific brain regions, as well as higher-order neural networks. Abnormal network activities may result in a vicious cycle, further impairing the integrity and functions of neurons and synapses, for example, through aberrant excitation or inhibition. Read More
Postepy Hig Med Dosw (Online) 2012 Apr 16;66:187-95. Epub 2012 Apr 16.
Katedra Biofizyki Ogólnej, Uniwersytet Łódzki.
All neurodegenerative diseases are related to pathology and accumulation of proteins. Proteins are basic structural and functional components of each cell and their functions are associated with their amino acid composition and spatial structure. The proper functioning of protein is necessary for the proper operation of the body system. Read More