Diverse Misfolded Conformational Strains and Cross-seeding of Misfolded Proteins Implicated in Neurodegenerative Diseases
نویسندگان
چکیده
منابع مشابه
Targeting Misfolded Proteins to Fight Neurodegenerative Diseases
With the assistance of proteins called chaperones, newly translated proteins fold into the three-dimensional shapes that are critical to their function. When proteins fold abnormally or become unstable, the consequences can be dire for cells, particularly neurons, which are exquisitely sensitive to the accumulation of misfolded proteins. Many neurodegenerative diseases, like Parkinson’s and Cre...
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Accumulation of misfolded protein aggregates is a hallmark event in diverse diseases. These structures are able to seed their own polymerization by acting as aggregation nuclei both in vitro and in vivo. Recent studies in animal models suggest that misfolded proteins associated with different diseases can synergize in a heterologous fashion, potentiating pathological mechanisms and accelerating...
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Inam Neuroscience Research Center, Department of Neurology, Sanbon Medical Center, Wonkwang University College of Medicine, Gunpo; Department of Neurology*, Seoul National University College of Medicine & Seoul National University Bundang Hospital, Seoul, Korea During recent years, there has been remarkable progress with respect to the identification of molecular mechanisms and underlying patho...
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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 proteaso...
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Formation, aggregation and transmission of abnormal proteins are common features in neurodegenerative disorders including Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis, and Huntington's disease. The mechanisms underlying protein alterations in neurodegenerative diseases remain controversial. Novel findings highlighted altered protein clearing systems as common biochemi...
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ژورنال
عنوان ژورنال: Frontiers in Molecular Neuroscience
سال: 2019
ISSN: 1662-5099
DOI: 10.3389/fnmol.2019.00158