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- W4282941984 endingPage "27" @default.
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- W4282941984 abstract "Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative disease that results from the loss of both upper and lower motor neurons. It is the most common motor neuron disease and currently has no effective treatment. There is mounting evidence to suggest that disturbances in proteostasis play a significant role in ALS pathogenesis. Proteostasis is the maintenance of the proteome at the right level, conformation and location to allow a cell to perform its intended function. In this review, we present a thorough synthesis of the literature that provides evidence that genetic mutations associated with ALS cause imbalance to a proteome that is vulnerable to such pressure due to its metastable nature. We propose that the mechanism underlying motor neuron death caused by defects in mRNA metabolism and protein degradation pathways converges on proteostasis dysfunction. We propose that the proteostasis network may provide an effective target for therapeutic development in ALS." @default.
- W4282941984 created "2022-06-16" @default.
- W4282941984 creator A5046298709 @default.
- W4282941984 creator A5075128023 @default.
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- W4282941984 date "2022-10-01" @default.
- W4282941984 modified "2023-09-27" @default.
- W4282941984 title "Proteostasis impairment and ALS" @default.
- W4282941984 cites W1143895086 @default.
- W4282941984 cites W148258150 @default.
- W4282941984 cites W1486608709 @default.
- W4282941984 cites W1489121534 @default.
- W4282941984 cites W1513849811 @default.
- W4282941984 cites W1525022089 @default.
- W4282941984 cites W1526095797 @default.
- W4282941984 cites W1535070554 @default.
- W4282941984 cites W1539814399 @default.
- W4282941984 cites W1542217606 @default.
- W4282941984 cites W1545244782 @default.
- W4282941984 cites W1549578642 @default.
- W4282941984 cites W1567632117 @default.
- W4282941984 cites W1619082318 @default.
- W4282941984 cites W1676877312 @default.
- W4282941984 cites W1908553983 @default.
- W4282941984 cites W1910374374 @default.
- W4282941984 cites W1922000354 @default.
- W4282941984 cites W1940436875 @default.
- W4282941984 cites W1963943740 @default.
- W4282941984 cites W1965286266 @default.
- W4282941984 cites W1965558349 @default.
- W4282941984 cites W1966202673 @default.
- W4282941984 cites W1966364136 @default.
- W4282941984 cites W1966398941 @default.
- W4282941984 cites W1967025628 @default.
- W4282941984 cites W1967443709 @default.
- W4282941984 cites W1967932847 @default.
- W4282941984 cites W1968109302 @default.
- W4282941984 cites W1968229731 @default.
- W4282941984 cites W1968892625 @default.
- W4282941984 cites W1969122757 @default.
- W4282941984 cites W1970506744 @default.
- W4282941984 cites W1971108880 @default.
- W4282941984 cites W1971144317 @default.
- W4282941984 cites W1972038271 @default.
- W4282941984 cites W1972121275 @default.
- W4282941984 cites W1972399884 @default.
- W4282941984 cites W1972434272 @default.
- W4282941984 cites W1973460029 @default.
- W4282941984 cites W1973851166 @default.
- W4282941984 cites W1974032274 @default.
- W4282941984 cites W1974396693 @default.
- W4282941984 cites W1974448552 @default.
- W4282941984 cites W1974809155 @default.
- W4282941984 cites W1976765363 @default.
- W4282941984 cites W1978911202 @default.
- W4282941984 cites W1979179514 @default.
- W4282941984 cites W1979937961 @default.
- W4282941984 cites W1979940972 @default.
- W4282941984 cites W1980128350 @default.
- W4282941984 cites W1980913885 @default.
- W4282941984 cites W1980939512 @default.
- W4282941984 cites W1981160857 @default.
- W4282941984 cites W1983633543 @default.
- W4282941984 cites W1984538960 @default.
- W4282941984 cites W1984678540 @default.
- W4282941984 cites W1985170292 @default.
- W4282941984 cites W1985171431 @default.
- W4282941984 cites W1985811907 @default.
- W4282941984 cites W1986846248 @default.
- W4282941984 cites W1986911150 @default.
- W4282941984 cites W1987066016 @default.
- W4282941984 cites W1987467454 @default.
- W4282941984 cites W1988146888 @default.
- W4282941984 cites W1988837475 @default.
- W4282941984 cites W1989332533 @default.
- W4282941984 cites W1989634299 @default.
- W4282941984 cites W1990145006 @default.
- W4282941984 cites W1990393476 @default.
- W4282941984 cites W1991570014 @default.
- W4282941984 cites W1991841644 @default.
- W4282941984 cites W1992002234 @default.
- W4282941984 cites W1994600281 @default.
- W4282941984 cites W1996037209 @default.
- W4282941984 cites W1996412713 @default.
- W4282941984 cites W1997369314 @default.
- W4282941984 cites W1999442278 @default.
- W4282941984 cites W1999630223 @default.
- W4282941984 cites W2001788248 @default.
- W4282941984 cites W2003395646 @default.
- W4282941984 cites W2003618511 @default.
- W4282941984 cites W2004178008 @default.
- W4282941984 cites W2004250476 @default.
- W4282941984 cites W2004480716 @default.
- W4282941984 cites W2004850573 @default.
- W4282941984 cites W2007239552 @default.
- W4282941984 cites W2008188499 @default.
- W4282941984 cites W2008316672 @default.
- W4282941984 cites W2008735323 @default.