PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 28359739-4 2017 Therefore, counteracting mutant huntingtin (mHtt)-induced oxidative stress and mitochondrial dysfunction appears as a new approach to treat this devastating disease. mhtt 44-48 huntingtin Mus musculus 32-42 33516737-3 2021 We previously described the 23aa Htt fragment P42, that attenuated the pathological phenotypes induced by mHtt. mhtt 106-110 huntingtin Mus musculus 33-36 26581643-23 2016 One of this was Hypk, a Htt-interacting chaperone protein with the ability to solubilize mHtt aggregated structures in cell lines. mhtt 89-93 huntingtin Mus musculus 24-27 25057205-5 2014 The serine/threonine kinase Akt, which regulates HTT function, rescued the spindle misorientation caused by the mHTT, by serine 421 (S421) phosphorylation, in cultured cells and in mice. mhtt 112-116 huntingtin Mus musculus 49-52 21542802-3 2011 To determine the extent of cell-autonomous effects of mhtt (mutant huntingtin) protein on vulnerability to excitotoxic insult in MSNs in vivo, we measured the number of degenerating neurons in response to intrastriatal injection of QA (quinolinic acid) in presymptomatic and symptomatic transgenic (D9-N171-98Q, also known as DE5) mice that express mhtt in MSNs but not in cortex. mhtt 54-58 huntingtin Mus musculus 67-77 20424159-1 2010 We recently reported that the small G-protein Rhes has the properties of a SUMO-E3 ligase and mediates mutant huntingtin (mHtt) cytotoxicity. mhtt 122-126 huntingtin Mus musculus 110-120 19864571-4 2009 We previously showed that V(L)12.3, an intrabody recognizing the N terminus of Htt, and Happ1, an intrabody recognizing the proline-rich domain of Htt, both reduce mHtt-induced toxicity and aggregation in cell culture and brain slice models of HD. mhtt 164-168 huntingtin Mus musculus 147-150 34884469-6 2021 Here, we tested the relevance of strain background for mutant huntingtin (mHTT) toxicity on the cellular level by investigating HD pathologies in YAC128 mice in the C57BL/6 background (YAC128/BL6). mhtt 74-78 huntingtin Mus musculus 62-72 32574176-1 2020 In the BACHD mouse model of Huntington"s disease (HD), deletion of the N17 domain of the Huntingtin gene (BACHDDeltaN17, Q97) has been reported to lead to nuclear accumulation of mHTT and exacerbation of motor deficits, neuroinflammation and striatal atrophy (Gu et al., 2015). mhtt 179-183 huntingtin Mus musculus 89-99 29151587-7 2017 Interestingly, their effects on HTT levels are mHTT-dependent, providing a feedback mechanism in which mHTT enhances its own level thus contributing to mHTT accumulation and disease progression. mhtt 47-51 huntingtin Mus musculus 32-35 29151587-7 2017 Interestingly, their effects on HTT levels are mHTT-dependent, providing a feedback mechanism in which mHTT enhances its own level thus contributing to mHTT accumulation and disease progression. mhtt 103-107 huntingtin Mus musculus 32-35 29151587-7 2017 Interestingly, their effects on HTT levels are mHTT-dependent, providing a feedback mechanism in which mHTT enhances its own level thus contributing to mHTT accumulation and disease progression. mhtt 103-107 huntingtin Mus musculus 32-35 33310753-5 2021 We have also shown that lowering HTT in the brains of HD mice results in correlative reduction of mHTT in the CSF, prompting the use of this measure as an exploratory marker of target engagement in clinical trials. mhtt 98-102 huntingtin Mus musculus 33-36 33310753-10 2021 Overall, our data supports both passive release and active clearance of mHTT into CSF, suggesting that its treatment-induced changes may represent a combination of target engagement and preservation of neurons.SIGNIFICANCE STATEMENTChanges in cerebrospinal fluid (CSF) mutant huntingtin (mHTT) are being used as an exploratory endpoint in HTT lowering clinical trials for the treatment of Huntington disease (HD). mhtt 72-76 huntingtin Mus musculus 276-286