PMC:6610326 / 60042-61635 JSONTXT

{"project":"2_test","denotations":[{"id":"31316328-17084815-38515675","span":{"begin":144,"end":148},"obj":"17084815"},{"id":"31316328-17469116-38515676","span":{"begin":168,"end":172},"obj":"17469116"},{"id":"31316328-17333220-38515677","span":{"begin":186,"end":190},"obj":"17333220"},{"id":"31316328-17786458-38515678","span":{"begin":206,"end":210},"obj":"17786458"},{"id":"31316328-19449021-38515679","span":{"begin":229,"end":233},"obj":"19449021"},{"id":"31316328-22083254-38515680","span":{"begin":250,"end":254},"obj":"22083254"},{"id":"31316328-23378219-38515681","span":{"begin":272,"end":276},"obj":"23378219"},{"id":"31316328-21604077-38515682","span":{"begin":421,"end":425},"obj":"21604077"},{"id":"31316328-23542689-38515683","span":{"begin":567,"end":571},"obj":"23542689"},{"id":"31316328-18176898-38515684","span":{"begin":923,"end":927},"obj":"18176898"},{"id":"31316328-23542689-38515685","span":{"begin":942,"end":946},"obj":"23542689"},{"id":"31316328-23378219-38515686","span":{"begin":964,"end":968},"obj":"23378219"},{"id":"31316328-18023016-38515687","span":{"begin":1166,"end":1170},"obj":"18023016"},{"id":"31316328-23378219-38515688","span":{"begin":1188,"end":1192},"obj":"23378219"},{"id":"31316328-28348221-38515689","span":{"begin":1287,"end":1291},"obj":"28348221"},{"id":"31316328-23542689-38515690","span":{"begin":1488,"end":1492},"obj":"23542689"}],"text":"In ALS, TDP-43 pathology has been detected in oligodendrocytes in the motor cortex and spinal cord of both SALS and FALS patients (Arai et al., 2006; Mackenzie et al., 2007; Tan et al., 2007; Zhang et al., 2008; Seilhean et al., 2009; Murray et al., 2011; Philips et al., 2013). In addition, FUS forms cytoplasmic aggregates in oligodendrocytes from ALS patients bearing FUSR521C or FUSP525L mutations (Mackenzie et al., 2011). Degeneration of oligodendrocytes and their precursors was also linked with axon demyelination in both SALS and FALS patients (Kang et al., 2013). In SOD1G93A mice, oligodendrocyte loss in the spinal cord occurs before symptoms appear and importantly, before MN loss, implying that oligodendrocytes are associated with disease onset. This MN loss increases with disease progression, resulting in MNs with only partially myelinated axons in SOD1G93A mice and SOD1G93A rats (Niebroj-Dobosz et al., 2007; Kang et al., 2013; Philips et al., 2013). Whilst the proliferation of oligodendrocyte precursors may compensate for this loss, newly synthetized oligodendrocytes failed to mature and remain dysfunctional in SOD1G93A mice (Magnus et al., 2008; Philips et al., 2013). Recently, SOD1G85R was able to transfer from MNs to nearby oligodendrocytes (Thomas et al., 2017). The selective removal of mutant SOD1 from NG2+ oligodendrocyte progenitors, but not mature oligodendrocytes in SOD1G37R mice, leads to delayed disease onset and prolonged survival (Kang et al., 2013), further suggesting that mutant SOD1-induced oligodendrocyte defects are detrimental to MNs in ALS."}

{"project":"0_colil","denotations":[{"id":"31316328-17084815-631132","span":{"begin":144,"end":148},"obj":"17084815"},{"id":"31316328-17469116-631133","span":{"begin":168,"end":172},"obj":"17469116"},{"id":"31316328-17333220-631134","span":{"begin":186,"end":190},"obj":"17333220"},{"id":"31316328-17786458-631135","span":{"begin":206,"end":210},"obj":"17786458"},{"id":"31316328-19449021-631136","span":{"begin":229,"end":233},"obj":"19449021"},{"id":"31316328-22083254-631137","span":{"begin":250,"end":254},"obj":"22083254"},{"id":"31316328-23378219-631138","span":{"begin":272,"end":276},"obj":"23378219"},{"id":"31316328-21604077-631139","span":{"begin":421,"end":425},"obj":"21604077"},{"id":"31316328-23542689-631140","span":{"begin":567,"end":571},"obj":"23542689"},{"id":"31316328-18176898-631141","span":{"begin":923,"end":927},"obj":"18176898"},{"id":"31316328-23542689-631142","span":{"begin":942,"end":946},"obj":"23542689"},{"id":"31316328-23378219-631143","span":{"begin":964,"end":968},"obj":"23378219"},{"id":"31316328-18023016-631144","span":{"begin":1166,"end":1170},"obj":"18023016"},{"id":"31316328-23378219-631145","span":{"begin":1188,"end":1192},"obj":"23378219"},{"id":"31316328-28348221-631146","span":{"begin":1287,"end":1291},"obj":"28348221"},{"id":"31316328-23542689-631147","span":{"begin":1488,"end":1492},"obj":"23542689"}],"text":"In ALS, TDP-43 pathology has been detected in oligodendrocytes in the motor cortex and spinal cord of both SALS and FALS patients (Arai et al., 2006; Mackenzie et al., 2007; Tan et al., 2007; Zhang et al., 2008; Seilhean et al., 2009; Murray et al., 2011; Philips et al., 2013). In addition, FUS forms cytoplasmic aggregates in oligodendrocytes from ALS patients bearing FUSR521C or FUSP525L mutations (Mackenzie et al., 2011). Degeneration of oligodendrocytes and their precursors was also linked with axon demyelination in both SALS and FALS patients (Kang et al., 2013). In SOD1G93A mice, oligodendrocyte loss in the spinal cord occurs before symptoms appear and importantly, before MN loss, implying that oligodendrocytes are associated with disease onset. This MN loss increases with disease progression, resulting in MNs with only partially myelinated axons in SOD1G93A mice and SOD1G93A rats (Niebroj-Dobosz et al., 2007; Kang et al., 2013; Philips et al., 2013). Whilst the proliferation of oligodendrocyte precursors may compensate for this loss, newly synthetized oligodendrocytes failed to mature and remain dysfunctional in SOD1G93A mice (Magnus et al., 2008; Philips et al., 2013). Recently, SOD1G85R was able to transfer from MNs to nearby oligodendrocytes (Thomas et al., 2017). The selective removal of mutant SOD1 from NG2+ oligodendrocyte progenitors, but not mature oligodendrocytes in SOD1G37R mice, leads to delayed disease onset and prolonged survival (Kang et al., 2013), further suggesting that mutant SOD1-induced oligodendrocyte defects are detrimental to MNs in ALS."}