PMC:4635215 / 14861-16130 JSONTXT

{"project":"TEST0","denotations":[{"id":"26594142-224-232-125982","span":{"begin":267,"end":271},"obj":"[\"24012756\"]"},{"id":"26594142-177-185-125983","span":{"begin":451,"end":455},"obj":"[\"16870622\"]"},{"id":"26594142-134-142-125984","span":{"begin":592,"end":596},"obj":"[\"21150128\"]"},{"id":"26594142-180-188-125985","span":{"begin":638,"end":642},"obj":"[\"25399611\"]"},{"id":"26594142-80-88-125986","span":{"begin":1118,"end":1122},"obj":"[\"14713958\"]"}],"text":"ACBD3 (also known as GCP60) is upregulated in cell culture models expressing the huntingtin (Htt) protein with expanded polyglutamine repeats, as well as in the brains of mice that model Huntington disease and in the striatum of Huntington's patients (Sbodio et al., 2013). ACBD3 is a ubiquitous peripheral Golgi protein that interacts with golgins and may regulate trafficking of fragments of golgin-160 generated by caspase cleavage (Sbodio et al., 2006). Interestingly, as mentioned above, ACBD3 is one of the genes shown to be upregulated by Golgi stress induced by monensin (Oku et al., 2011), which requires TFE3 (Taniguchi et al., 2015). In striatal neurons, increased levels of ACBD3 may lead to increased neurotoxicity due to its interaction with both Rhes and Htt. Rhes is a small G protein that is associated with Htt pathogenicity. Rhes is specifically expressed in the striatum (unlike Htt, which is widely expressed) and is thus thought to limit the site of degeneration in Huntington's disease to this region of the brain. Caspase-2 cleavage of Htt has also been implicated in pathology (Hermel et al., 2004). It will be interesting to see if any other binding partners of ACBD3 and caspase-2 cleavage at the Golgi are involved in Huntington's pathology."}

{"project":"0_colil","denotations":[{"id":"26594142-24012756-125982","span":{"begin":267,"end":271},"obj":"24012756"},{"id":"26594142-16870622-125983","span":{"begin":451,"end":455},"obj":"16870622"},{"id":"26594142-21150128-125984","span":{"begin":592,"end":596},"obj":"21150128"},{"id":"26594142-25399611-125985","span":{"begin":638,"end":642},"obj":"25399611"},{"id":"26594142-14713958-125986","span":{"begin":1118,"end":1122},"obj":"14713958"}],"text":"ACBD3 (also known as GCP60) is upregulated in cell culture models expressing the huntingtin (Htt) protein with expanded polyglutamine repeats, as well as in the brains of mice that model Huntington disease and in the striatum of Huntington's patients (Sbodio et al., 2013). ACBD3 is a ubiquitous peripheral Golgi protein that interacts with golgins and may regulate trafficking of fragments of golgin-160 generated by caspase cleavage (Sbodio et al., 2006). Interestingly, as mentioned above, ACBD3 is one of the genes shown to be upregulated by Golgi stress induced by monensin (Oku et al., 2011), which requires TFE3 (Taniguchi et al., 2015). In striatal neurons, increased levels of ACBD3 may lead to increased neurotoxicity due to its interaction with both Rhes and Htt. Rhes is a small G protein that is associated with Htt pathogenicity. Rhes is specifically expressed in the striatum (unlike Htt, which is widely expressed) and is thus thought to limit the site of degeneration in Huntington's disease to this region of the brain. Caspase-2 cleavage of Htt has also been implicated in pathology (Hermel et al., 2004). It will be interesting to see if any other binding partners of ACBD3 and caspase-2 cleavage at the Golgi are involved in Huntington's pathology."}

{"project":"2_test","denotations":[{"id":"26594142-24012756-38084454","span":{"begin":267,"end":271},"obj":"24012756"},{"id":"26594142-16870622-38084455","span":{"begin":451,"end":455},"obj":"16870622"},{"id":"26594142-21150128-38084456","span":{"begin":592,"end":596},"obj":"21150128"},{"id":"26594142-25399611-38084457","span":{"begin":638,"end":642},"obj":"25399611"},{"id":"26594142-14713958-38084458","span":{"begin":1118,"end":1122},"obj":"14713958"}],"text":"ACBD3 (also known as GCP60) is upregulated in cell culture models expressing the huntingtin (Htt) protein with expanded polyglutamine repeats, as well as in the brains of mice that model Huntington disease and in the striatum of Huntington's patients (Sbodio et al., 2013). ACBD3 is a ubiquitous peripheral Golgi protein that interacts with golgins and may regulate trafficking of fragments of golgin-160 generated by caspase cleavage (Sbodio et al., 2006). Interestingly, as mentioned above, ACBD3 is one of the genes shown to be upregulated by Golgi stress induced by monensin (Oku et al., 2011), which requires TFE3 (Taniguchi et al., 2015). In striatal neurons, increased levels of ACBD3 may lead to increased neurotoxicity due to its interaction with both Rhes and Htt. Rhes is a small G protein that is associated with Htt pathogenicity. Rhes is specifically expressed in the striatum (unlike Htt, which is widely expressed) and is thus thought to limit the site of degeneration in Huntington's disease to this region of the brain. Caspase-2 cleavage of Htt has also been implicated in pathology (Hermel et al., 2004). It will be interesting to see if any other binding partners of ACBD3 and caspase-2 cleavage at the Golgi are involved in Huntington's pathology."}