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- W2001838312 abstract "Inam Neuroscience Research Center, Department of Neurology, Sanbon Medical Center, College of Medicine, Wonkwang University, Gunpo, Korea The majority of neurodegenerative dementias are thought to result primarily from the misfolding, aggregation and accumulation of proteins which interfere with protein homeostasis in the brain. Some of them are caused by the expansion of unstable nucleotide repeats, which include Huntington’s disease as a prototype. Other neurodevelopmental or neurodegenerative disorders, such as fragile X syndrome, some spinocerebellar ataxias and myotonic dystrophies exhibit cognitive or behavioral deficits as parts of their clinical manifestations. Unstable repeat expansions include trinucleotide, tetranucleotide, and pentanucleotide. Recently hexanucleotide repeat expansion in frontotemporal dementia and amyotrophic lateral sclerosis was identified. The pathogenic mechanisms for these repeat disorders include either loss of protein function or gain of function at the protein or RNA levels. The aim of this article is to review proposed mechanisms by which unstable repeat expansions give rise to degeneration of brain with the hope of understanding the diseases and providing insights into the areas of therapeutic intervention. We will review these potential mechanisms in the context of fragile X syndrome, Huntington’s disease, spinocerebellar ataxias, myotonic dystrophy, and frontotemporal dementia and amyotrophic lateral sclerosis. We will also discuss the potential targets for therapeutic intervention. Received: February 17, 2011 Revision received: March 7, 2011 Accepted: March 7, 2011" @default.
- W2001838312 created "2016-06-24" @default.
- W2001838312 creator A5011134183 @default.
- W2001838312 creator A5035768302 @default.
- W2001838312 creator A5086083933 @default.
- W2001838312 date "2012-01-01" @default.
- W2001838312 modified "2023-09-23" @default.
- W2001838312 title "Unstable Repeat Expansion in Neurodegenerative Dementias: Mechanisms of Disease" @default.
- W2001838312 cites W1573542653 @default.
- W2001838312 cites W1575708112 @default.
- W2001838312 cites W1642338065 @default.
- W2001838312 cites W1708445842 @default.
- W2001838312 cites W1858416803 @default.
- W2001838312 cites W1967630695 @default.
- W2001838312 cites W1968327772 @default.
- W2001838312 cites W1968874378 @default.
- W2001838312 cites W1972453061 @default.
- W2001838312 cites W1977627907 @default.
- W2001838312 cites W1978104052 @default.
- W2001838312 cites W1983792907 @default.
- W2001838312 cites W1985474993 @default.
- W2001838312 cites W1986022352 @default.
- W2001838312 cites W1987286446 @default.
- W2001838312 cites W1988283896 @default.
- W2001838312 cites W1988498459 @default.
- W2001838312 cites W1988645307 @default.
- W2001838312 cites W1990059209 @default.
- W2001838312 cites W1992609622 @default.
- W2001838312 cites W1994674028 @default.
- W2001838312 cites W1995058974 @default.
- W2001838312 cites W1997555550 @default.
- W2001838312 cites W2003054268 @default.
- W2001838312 cites W2004901342 @default.
- W2001838312 cites W2005078842 @default.
- W2001838312 cites W2007157911 @default.
- W2001838312 cites W2009002061 @default.
- W2001838312 cites W2010997388 @default.
- W2001838312 cites W2012481532 @default.
- W2001838312 cites W2019417406 @default.
- W2001838312 cites W2025413109 @default.
- W2001838312 cites W2029948374 @default.
- W2001838312 cites W2030824896 @default.
- W2001838312 cites W2031785254 @default.
- W2001838312 cites W2032844625 @default.
- W2001838312 cites W2036872469 @default.
- W2001838312 cites W2038306358 @default.
- W2001838312 cites W2039214189 @default.
- W2001838312 cites W2042567578 @default.
- W2001838312 cites W2042739691 @default.
- W2001838312 cites W2045753346 @default.
- W2001838312 cites W2050607203 @default.
- W2001838312 cites W2061370790 @default.
- W2001838312 cites W2063685191 @default.
- W2001838312 cites W2064759892 @default.
- W2001838312 cites W2067439875 @default.
- W2001838312 cites W2068422066 @default.
- W2001838312 cites W2074525195 @default.
- W2001838312 cites W2078217107 @default.
- W2001838312 cites W2078508295 @default.
- W2001838312 cites W2080828566 @default.
- W2001838312 cites W2081280332 @default.
- W2001838312 cites W2081748748 @default.
- W2001838312 cites W2084091637 @default.
- W2001838312 cites W2084889756 @default.
- W2001838312 cites W2088489316 @default.
- W2001838312 cites W2095324056 @default.
- W2001838312 cites W2096195752 @default.
- W2001838312 cites W2099039583 @default.
- W2001838312 cites W2101042581 @default.
- W2001838312 cites W2101787765 @default.
- W2001838312 cites W2103116226 @default.
- W2001838312 cites W2105954114 @default.
- W2001838312 cites W2106490406 @default.
- W2001838312 cites W2108780282 @default.
- W2001838312 cites W2109436341 @default.
- W2001838312 cites W2110640321 @default.
- W2001838312 cites W2112072636 @default.
- W2001838312 cites W2113998330 @default.
- W2001838312 cites W2115110458 @default.
- W2001838312 cites W2116276594 @default.
- W2001838312 cites W2119080469 @default.
- W2001838312 cites W2119194938 @default.
- W2001838312 cites W2125608178 @default.
- W2001838312 cites W2125931517 @default.
- W2001838312 cites W2126880574 @default.
- W2001838312 cites W2129134595 @default.
- W2001838312 cites W2129698270 @default.
- W2001838312 cites W2131173813 @default.
- W2001838312 cites W2131472300 @default.
- W2001838312 cites W2132852866 @default.
- W2001838312 cites W2135489189 @default.
- W2001838312 cites W2136949432 @default.
- W2001838312 cites W2139869327 @default.
- W2001838312 cites W2139919925 @default.
- W2001838312 cites W2140188816 @default.
- W2001838312 cites W2140312446 @default.
- W2001838312 cites W2140382995 @default.
- W2001838312 cites W2143106924 @default.