FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2019954524> ?p ?o ?g. }

• W2019954524 endingPage "20" @default.
• W2019954524 startingPage "20" @default.
• W2019954524 abstract "Despite enormous progress in elucidating the biophysics of aggregation, no cause-and-effect relationship between protein aggregation and neurodegenerative disease has been unequivocally established. Here, we derived several risk-based stochastic kinetic models that assess genotype/phenotype correlations in patients with Huntington’s disease (HD) caused by the expansion of a CAG repeat. Fascinating disease-specific aspects of HD include the polyglutamine (polyQ)-length dependence of both age at symptoms onset and the propensity of the expanded polyQ protein to aggregate. In vitro, aggregation of polyQ peptides follows a simple nucleated growth polymerization pathway. Our models that reflect polyQ aggregation kinetics in a nucleated growth polymerization divided aggregate process into the length-dependent nucleation and the nucleation-dependent elongation. In contrast to the repeat-length dependent variability of age at onset, recent studies have shown that the extent of expansion has only a subtle effect on the rate of disease progression, suggesting possible differences in the mechanisms underlying the neurodegenerative process. Using polyQ-length as an index, these procedures enabled us for the first time to establish a quantitative connection between aggregation kinetics and disease process, including onset and the rate of progression. Although the complexity of disease process in HD, the time course of striatal neurodegeneration can be precisely predicted by the mathematical model in which neurodegeneration occurs by different mechanisms for the initiation and progression of disease processes. Nucleation is sufficient to initiate neuronal loss as a series of random events in time. The stochastic appearance of nucleation in a cell population acts as the constant risk of neuronal cell damage over time, while elongation reduces the risk by nucleation in proportion to the increased extent of the aggregates during disease progression. Our findings suggest that nucleation is a critical step in gaining toxic effects to the cell, and provide a new insight into the relationship between polyQ aggregation and neurodegenerative process in HD." @default.
• W2019954524 created "2016-06-24" @default.
• W2019954524 creator A5051302366 @default.
• W2019954524 creator A5070170460 @default.
• W2019954524 date "2012-01-01" @default.
• W2019954524 modified "2023-09-23" @default.
• W2019954524 title "Quantitative connection between polyglutamine aggregation kinetics and neurodegenerative process in patients with Huntington’s disease" @default.
• W2019954524 cites W1504186711 @default.
• W2019954524 cites W1544063520 @default.
• W2019954524 cites W1548441884 @default.
• W2019954524 cites W1967015107 @default.
• W2019954524 cites W1970456057 @default.
• W2019954524 cites W1973055895 @default.
• W2019954524 cites W1976345822 @default.
• W2019954524 cites W1979964642 @default.
• W2019954524 cites W1980825524 @default.
• W2019954524 cites W1982138826 @default.
• W2019954524 cites W1983789627 @default.
• W2019954524 cites W1992035131 @default.
• W2019954524 cites W1995008214 @default.
• W2019954524 cites W2001573546 @default.
• W2019954524 cites W2006298998 @default.
• W2019954524 cites W2008307658 @default.
• W2019954524 cites W2015789019 @default.
• W2019954524 cites W2016293448 @default.
• W2019954524 cites W2021211601 @default.
• W2019954524 cites W2025295962 @default.
• W2019954524 cites W2025578163 @default.
• W2019954524 cites W2025851491 @default.
• W2019954524 cites W2030985020 @default.
• W2019954524 cites W2032730097 @default.
• W2019954524 cites W2036563840 @default.
• W2019954524 cites W2041416533 @default.
• W2019954524 cites W2048599819 @default.
• W2019954524 cites W2069452881 @default.
• W2019954524 cites W2073012734 @default.
• W2019954524 cites W2095529469 @default.
• W2019954524 cites W2101101654 @default.
• W2019954524 cites W2103782264 @default.
• W2019954524 cites W2106200048 @default.
• W2019954524 cites W2114289597 @default.
• W2019954524 cites W2115396351 @default.
• W2019954524 cites W2121852043 @default.
• W2019954524 cites W2121963977 @default.
• W2019954524 cites W2123544060 @default.
• W2019954524 cites W2129167248 @default.
• W2019954524 cites W2135269664 @default.
• W2019954524 cites W2139412695 @default.
• W2019954524 cites W2153754131 @default.
• W2019954524 cites W2160612991 @default.
• W2019954524 cites W2163671213 @default.
• W2019954524 cites W2165317828 @default.
• W2019954524 cites W2165575517 @default.
• W2019954524 cites W2165723232 @default.
• W2019954524 cites W89892064 @default.
• W2019954524 doi "https://doi.org/10.1186/1750-1326-7-20" @default.
• W2019954524 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3468392" @default.
• W2019954524 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/22583646" @default.
• W2019954524 hasPublicationYear "2012" @default.
• W2019954524 type Work @default.
• W2019954524 sameAs 2019954524 @default.
• W2019954524 citedByCount "4" @default.
• W2019954524 countsByYear W20199545242012 @default.
• W2019954524 countsByYear W20199545242014 @default.
• W2019954524 countsByYear W20199545242017 @default.
• W2019954524 crossrefType "journal-article" @default.
• W2019954524 hasAuthorship W2019954524A5051302366 @default.
• W2019954524 hasAuthorship W2019954524A5070170460 @default.
• W2019954524 hasBestOaLocation W20199545241 @default.
• W2019954524 hasConcept C136238340 @default.
• W2019954524 hasConcept C142724271 @default.
• W2019954524 hasConcept C169760540 @default.
• W2019954524 hasConcept C178790620 @default.
• W2019954524 hasConcept C185592680 @default.
• W2019954524 hasConcept C2776925932 @default.
• W2019954524 hasConcept C2779134260 @default.
• W2019954524 hasConcept C2780647506 @default.
• W2019954524 hasConcept C2908647359 @default.
• W2019954524 hasConcept C54355233 @default.
• W2019954524 hasConcept C61048295 @default.
• W2019954524 hasConcept C71924100 @default.
• W2019954524 hasConcept C86803240 @default.
• W2019954524 hasConcept C99454951 @default.
• W2019954524 hasConceptScore W2019954524C136238340 @default.
• W2019954524 hasConceptScore W2019954524C142724271 @default.
• W2019954524 hasConceptScore W2019954524C169760540 @default.
• W2019954524 hasConceptScore W2019954524C178790620 @default.
• W2019954524 hasConceptScore W2019954524C185592680 @default.
• W2019954524 hasConceptScore W2019954524C2776925932 @default.
• W2019954524 hasConceptScore W2019954524C2779134260 @default.
• W2019954524 hasConceptScore W2019954524C2780647506 @default.
• W2019954524 hasConceptScore W2019954524C2908647359 @default.
• W2019954524 hasConceptScore W2019954524C54355233 @default.
• W2019954524 hasConceptScore W2019954524C61048295 @default.
• W2019954524 hasConceptScore W2019954524C71924100 @default.
• W2019954524 hasConceptScore W2019954524C86803240 @default.
• W2019954524 hasConceptScore W2019954524C99454951 @default.
• W2019954524 hasIssue "1" @default.

• next