PMC:6610326 / 1317-3053
Annnotations
{"target":"https://pubannotation.org/docs/sourcedb/PMC/sourceid/6610326","sourcedb":"PMC","sourceid":"6610326","source_url":"https://www.ncbi.nlm.nih.gov/pmc/6610326","text":"Amyotrophic lateral sclerosis (ALS) is a late-onset, progressive and fatal neurodegenerative disease which primarily affects motor neurons (MNs) of the motor cortex of the brain, brainstem motor nuclei and anterior horn of the spinal cord (Kiernan et al., 2011; Renton et al., 2014; Al Sultan et al., 2016; Taylor et al., 2016). ALS commonly begins in late-adulthood, when patients first experience focal symptoms, such as weakness in the limb or bulbar muscles, as well as widespread fasciculations. The disease then usually progresses in an organized way to adjacent areas of the central nervous system (CNS), and consequently symptoms appear in other regions of the body. Several clinical subsets of ALS can be distinguished by the anatomical location first affected (Renton et al., 2014; Taylor et al., 2016). This includes bulbar onset, where symptoms first appear in the muscles controlling speech, mastication and swallowing; and limb onset, where symptoms present initially in the upper (arm or hand) or lower limbs (leg or foot). Bulbar onset patients face a much worse prognosis than those with spinal onset ALS, where the average survival time following diagnosis is less than 2 years. However, in patients with the much rarer respiratory onset form (3–5%), the prognosis is even worse as the survival time following diagnosis is only 1.4 years (Swinnen and Robberecht, 2014). At disease end stage, only support and palliation are available, and patients usually die from respiratory failure, typically 3–5 years after diagnosis (Taylor et al., 2016). There are currently few effective treatments. Hence there is an urgent need to understand the underlying causes and risk factors for ALS to discover new therapeutic targets.","tracks":[{"project":"2_test","denotations":[{"id":"31316328-21296405-38515268","span":{"begin":256,"end":260},"obj":"21296405"},{"id":"31316328-24369373-38515269","span":{"begin":277,"end":281},"obj":"24369373"},{"id":"31316328-27830784-38515270","span":{"begin":322,"end":326},"obj":"27830784"},{"id":"31316328-24369373-38515271","span":{"begin":786,"end":790},"obj":"24369373"},{"id":"31316328-27830784-38515272","span":{"begin":807,"end":811},"obj":"27830784"},{"id":"31316328-25311585-38515273","span":{"begin":1381,"end":1385},"obj":"25311585"},{"id":"31316328-27830784-38515274","span":{"begin":1556,"end":1560},"obj":"27830784"}],"attributes":[{"subj":"31316328-21296405-38515268","pred":"source","obj":"2_test"},{"subj":"31316328-24369373-38515269","pred":"source","obj":"2_test"},{"subj":"31316328-27830784-38515270","pred":"source","obj":"2_test"},{"subj":"31316328-24369373-38515271","pred":"source","obj":"2_test"},{"subj":"31316328-27830784-38515272","pred":"source","obj":"2_test"},{"subj":"31316328-25311585-38515273","pred":"source","obj":"2_test"},{"subj":"31316328-27830784-38515274","pred":"source","obj":"2_test"}]},{"project":"0_colil","denotations":[{"id":"31316328-21296405-630725","span":{"begin":256,"end":260},"obj":"21296405"},{"id":"31316328-24369373-630726","span":{"begin":277,"end":281},"obj":"24369373"},{"id":"31316328-27830784-630727","span":{"begin":322,"end":326},"obj":"27830784"},{"id":"31316328-24369373-630728","span":{"begin":786,"end":790},"obj":"24369373"},{"id":"31316328-27830784-630729","span":{"begin":807,"end":811},"obj":"27830784"},{"id":"31316328-25311585-630730","span":{"begin":1381,"end":1385},"obj":"25311585"},{"id":"31316328-27830784-630731","span":{"begin":1556,"end":1560},"obj":"27830784"}],"attributes":[{"subj":"31316328-21296405-630725","pred":"source","obj":"0_colil"},{"subj":"31316328-24369373-630726","pred":"source","obj":"0_colil"},{"subj":"31316328-27830784-630727","pred":"source","obj":"0_colil"},{"subj":"31316328-24369373-630728","pred":"source","obj":"0_colil"},{"subj":"31316328-27830784-630729","pred":"source","obj":"0_colil"},{"subj":"31316328-25311585-630730","pred":"source","obj":"0_colil"},{"subj":"31316328-27830784-630731","pred":"source","obj":"0_colil"}]}],"config":{"attribute types":[{"pred":"source","value type":"selection","values":[{"id":"2_test","color":"#da93ec","default":true},{"id":"0_colil","color":"#93ecc0"}]}]}}