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- W2189128975 abstract "Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease, which primarily affects motor neurons leading to progressive paralysis and death within a few years from onset. The pathological hallmark of ALS is the presence of cytoplasmic ubiquitinated protein inclusions in motor neurons and glial cells primarily in the spinal cord. While the vast majority of ALS occurs sporadically (sALS), in ~10% of cases, called familial ALS (fALS), there is clear indication of genetic inheritance. In the last decade, enormous progress was made in unravelling the aetiology of the disease, with the identification of ALS-causing mutations in new genes, as well as key molecular players involved in the origin or progression of ALS. However, much more needs to be done, as the pathogenic mechanisms triggered by a genetic or sporadic event leading to cytotoxicity and neuronal cell death are still poorly understood. The recent discoveries offer new possibilities for devising experimental animal and cellular models, which will hopefully contribute to the development of new techniques for early diagnosis and the identification of therapeutic targets for ALS. Here we review the current understanding of the aetiology, genetics, and pathogenic factors and mechanisms of ALS. We also discuss the challenges in deciphering ALS pathogenesis that result from the high complexity and heterogeneity of the disease." @default.
- W2189128975 created "2016-06-24" @default.
- W2189128975 creator A5075904616 @default.
- W2189128975 creator A5080687016 @default.
- W2189128975 date "2015-01-30" @default.
- W2189128975 modified "2023-09-27" @default.
- W2189128975 title "Advances and challenges in understanding the multifaceted pathogenesis of amyotrophic lateral sclerosis" @default.
- W2189128975 cites W107021871 @default.
- W2189128975 cites W1525431743 @default.
- W2189128975 cites W1619082318 @default.
- W2189128975 cites W1927588525 @default.
- W2189128975 cites W1963767682 @default.
- W2189128975 cites W1965095016 @default.
- W2189128975 cites W1965558349 @default.
- W2189128975 cites W1967577586 @default.
- W2189128975 cites W1967851074 @default.
- W2189128975 cites W1968758728 @default.
- W2189128975 cites W1977399503 @default.
- W2189128975 cites W1979535442 @default.
- W2189128975 cites W1984308849 @default.
- W2189128975 cites W1984538960 @default.
- W2189128975 cites W1991570014 @default.
- W2189128975 cites W1995185770 @default.
- W2189128975 cites W1999899839 @default.
- W2189128975 cites W2003618511 @default.
- W2189128975 cites W2004415828 @default.
- W2189128975 cites W2007239552 @default.
- W2189128975 cites W2007568107 @default.
- W2189128975 cites W2011396520 @default.
- W2189128975 cites W2012851706 @default.
- W2189128975 cites W2013131112 @default.
- W2189128975 cites W2013135033 @default.
- W2189128975 cites W2013488820 @default.
- W2189128975 cites W2016332933 @default.
- W2189128975 cites W2017062988 @default.
- W2189128975 cites W2017199287 @default.
- W2189128975 cites W2020159880 @default.
- W2189128975 cites W2024059905 @default.
- W2189128975 cites W2025961979 @default.
- W2189128975 cites W2031964319 @default.
- W2189128975 cites W2032719480 @default.
- W2189128975 cites W2033158384 @default.
- W2189128975 cites W2036693225 @default.
- W2189128975 cites W2038735530 @default.
- W2189128975 cites W2042396829 @default.
- W2189128975 cites W2042825905 @default.
- W2189128975 cites W2042930414 @default.
- W2189128975 cites W2045708478 @default.
- W2189128975 cites W2046095963 @default.
- W2189128975 cites W2047517574 @default.
- W2189128975 cites W2047867249 @default.
- W2189128975 cites W2053362200 @default.
- W2189128975 cites W2053863700 @default.
- W2189128975 cites W2054811674 @default.
- W2189128975 cites W2057170892 @default.
- W2189128975 cites W2060433067 @default.
- W2189128975 cites W2063198655 @default.
- W2189128975 cites W2063996210 @default.
- W2189128975 cites W2065219073 @default.
- W2189128975 cites W2066002405 @default.
- W2189128975 cites W2066828509 @default.
- W2189128975 cites W2066874059 @default.
- W2189128975 cites W2068147651 @default.
- W2189128975 cites W2068966740 @default.
- W2189128975 cites W2069045437 @default.
- W2189128975 cites W2069171272 @default.
- W2189128975 cites W2069350252 @default.
- W2189128975 cites W2070086306 @default.
- W2189128975 cites W2071471772 @default.
- W2189128975 cites W2072413533 @default.
- W2189128975 cites W2072978915 @default.
- W2189128975 cites W2073073462 @default.
- W2189128975 cites W2073190029 @default.
- W2189128975 cites W2074427781 @default.
- W2189128975 cites W2074726646 @default.
- W2189128975 cites W2075186362 @default.
- W2189128975 cites W2075588816 @default.
- W2189128975 cites W2076047037 @default.
- W2189128975 cites W2076495609 @default.
- W2189128975 cites W2079470515 @default.
- W2189128975 cites W2080964315 @default.
- W2189128975 cites W2084561728 @default.
- W2189128975 cites W2084942172 @default.
- W2189128975 cites W2086406144 @default.
- W2189128975 cites W2088856774 @default.
- W2189128975 cites W2090365926 @default.
- W2189128975 cites W2090382713 @default.
- W2189128975 cites W2092571154 @default.
- W2189128975 cites W2093394063 @default.
- W2189128975 cites W2093626853 @default.
- W2189128975 cites W2093933725 @default.
- W2189128975 cites W2096386031 @default.
- W2189128975 cites W2097228496 @default.
- W2189128975 cites W2098022922 @default.
- W2189128975 cites W2099340107 @default.
- W2189128975 cites W2101042581 @default.
- W2189128975 cites W2101687573 @default.
- W2189128975 cites W2102327538 @default.
- W2189128975 cites W2104384276 @default.
- W2189128975 cites W2106000656 @default.