PMC:6610326 / 99529-100633
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":"Axonal transport defects may be an important factor underlying the selective vulnerability of MNs or MN subtypes in ALS/FTD. Abnormal accumulation of phosphorylated neurofilaments, mitochondria and lysosomes in the proximal axon of large MNs and axonal spheroids, are present in SALS and FALS patients (Hirano et al., 1984; Corbo and Hays, 1992; Okada et al., 1995; Rouleau et al., 1996; Sasaki and Iwata, 1996). Mutant SOD1 slows both anterograde (Williamson and Cleveland, 1999) and retrograde (Chen et al., 2007; Perlson et al., 2009) axonal transport. Cytoskeletal and motor proteins are differentially expressed in spinal MNs compared to oculomotor neurons. This includes peripherin (Hedlund et al., 2010; Comley et al., 2015), which is also found in ubiquitinated inclusions in the spinal cord of FALS (Robertson et al., 2003) and SALS patients (He and Hays, 2004). Overexpression of peripherin leads to defective axonal transport (Millecamps et al., 2006) and late-onset MN degeneration (Beaulieu et al., 1999), implying that differential expression of peripherin contributes to neurodegeneration.","tracks":[{"project":"2_test","denotations":[{"id":"31316328-6540800-38515943","span":{"begin":318,"end":322},"obj":"6540800"},{"id":"31316328-1381416-38515944","span":{"begin":340,"end":344},"obj":"1381416"},{"id":"31316328-7539720-38515945","span":{"begin":360,"end":364},"obj":"7539720"},{"id":"31316328-8572658-38515946","span":{"begin":382,"end":386},"obj":"8572658"},{"id":"31316328-8834540-38515947","span":{"begin":406,"end":410},"obj":"8834540"},{"id":"31316328-10195180-38515948","span":{"begin":475,"end":479},"obj":"10195180"},{"id":"31316328-18160659-38515949","span":{"begin":510,"end":514},"obj":"18160659"},{"id":"31316328-19657041-38515950","span":{"begin":532,"end":536},"obj":"19657041"},{"id":"31316328-20826431-38515951","span":{"begin":705,"end":709},"obj":"20826431"},{"id":"31316328-25697826-38515952","span":{"begin":726,"end":730},"obj":"25697826"},{"id":"31316328-12642616-38515953","span":{"begin":827,"end":831},"obj":"12642616"},{"id":"31316328-14675609-38515954","span":{"begin":865,"end":869},"obj":"14675609"},{"id":"31316328-16787413-38515955","span":{"begin":957,"end":961},"obj":"16787413"},{"id":"31316328-15132161-38515956","span":{"begin":1012,"end":1016},"obj":"15132161"}],"attributes":[{"subj":"31316328-6540800-38515943","pred":"source","obj":"2_test"},{"subj":"31316328-1381416-38515944","pred":"source","obj":"2_test"},{"subj":"31316328-7539720-38515945","pred":"source","obj":"2_test"},{"subj":"31316328-8572658-38515946","pred":"source","obj":"2_test"},{"subj":"31316328-8834540-38515947","pred":"source","obj":"2_test"},{"subj":"31316328-10195180-38515948","pred":"source","obj":"2_test"},{"subj":"31316328-18160659-38515949","pred":"source","obj":"2_test"},{"subj":"31316328-19657041-38515950","pred":"source","obj":"2_test"},{"subj":"31316328-20826431-38515951","pred":"source","obj":"2_test"},{"subj":"31316328-25697826-38515952","pred":"source","obj":"2_test"},{"subj":"31316328-12642616-38515953","pred":"source","obj":"2_test"},{"subj":"31316328-14675609-38515954","pred":"source","obj":"2_test"},{"subj":"31316328-16787413-38515955","pred":"source","obj":"2_test"},{"subj":"31316328-15132161-38515956","pred":"source","obj":"2_test"}]},{"project":"0_colil","denotations":[{"id":"31316328-6540800-631400","span":{"begin":318,"end":322},"obj":"6540800"},{"id":"31316328-1381416-631401","span":{"begin":340,"end":344},"obj":"1381416"},{"id":"31316328-7539720-631402","span":{"begin":360,"end":364},"obj":"7539720"},{"id":"31316328-8572658-631403","span":{"begin":382,"end":386},"obj":"8572658"},{"id":"31316328-8834540-631404","span":{"begin":406,"end":410},"obj":"8834540"},{"id":"31316328-10195180-631405","span":{"begin":475,"end":479},"obj":"10195180"},{"id":"31316328-18160659-631406","span":{"begin":510,"end":514},"obj":"18160659"},{"id":"31316328-19657041-631407","span":{"begin":532,"end":536},"obj":"19657041"},{"id":"31316328-20826431-631408","span":{"begin":705,"end":709},"obj":"20826431"},{"id":"31316328-25697826-631409","span":{"begin":726,"end":730},"obj":"25697826"},{"id":"31316328-12642616-631410","span":{"begin":827,"end":831},"obj":"12642616"},{"id":"31316328-14675609-631411","span":{"begin":865,"end":869},"obj":"14675609"},{"id":"31316328-16787413-631412","span":{"begin":957,"end":961},"obj":"16787413"},{"id":"31316328-15132161-631413","span":{"begin":1012,"end":1016},"obj":"15132161"}],"attributes":[{"subj":"31316328-6540800-631400","pred":"source","obj":"0_colil"},{"subj":"31316328-1381416-631401","pred":"source","obj":"0_colil"},{"subj":"31316328-7539720-631402","pred":"source","obj":"0_colil"},{"subj":"31316328-8572658-631403","pred":"source","obj":"0_colil"},{"subj":"31316328-8834540-631404","pred":"source","obj":"0_colil"},{"subj":"31316328-10195180-631405","pred":"source","obj":"0_colil"},{"subj":"31316328-18160659-631406","pred":"source","obj":"0_colil"},{"subj":"31316328-19657041-631407","pred":"source","obj":"0_colil"},{"subj":"31316328-20826431-631408","pred":"source","obj":"0_colil"},{"subj":"31316328-25697826-631409","pred":"source","obj":"0_colil"},{"subj":"31316328-12642616-631410","pred":"source","obj":"0_colil"},{"subj":"31316328-14675609-631411","pred":"source","obj":"0_colil"},{"subj":"31316328-16787413-631412","pred":"source","obj":"0_colil"},{"subj":"31316328-15132161-631413","pred":"source","obj":"0_colil"}]}],"config":{"attribute types":[{"pred":"source","value type":"selection","values":[{"id":"2_test","color":"#ebec93","default":true},{"id":"0_colil","color":"#d293ec"}]}]}}