PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 18753376-1 2008 Huntington"s disease (HD) is a polyglutamine [poly(Q)] disease with an expanded poly(Q) stretch in the N terminus of the huntingtin protein (htt). polyglutamine 31-44 huntingtin Rattus norvegicus 121-131 19352548-1 2009 Huntington"s disease (HD) is caused by an expansion of polyglutamine tract in N-terminus of huntingtin (htt). polyglutamine 55-68 huntingtin Rattus norvegicus 92-102 19352548-1 2009 Huntington"s disease (HD) is caused by an expansion of polyglutamine tract in N-terminus of huntingtin (htt). polyglutamine 55-68 huntingtin Rattus norvegicus 104-107 19022249-1 2009 Huntington"s disease (HD) is a neurodegenerative disorder resulting from the expansion of a glutamine repeat (polyQ) in the N-terminus of the huntingtin (htt) protein. polyglutamine 110-115 huntingtin Rattus norvegicus 142-152 19022249-1 2009 Huntington"s disease (HD) is a neurodegenerative disorder resulting from the expansion of a glutamine repeat (polyQ) in the N-terminus of the huntingtin (htt) protein. polyglutamine 110-115 huntingtin Rattus norvegicus 154-157 19015277-5 2008 We further decipher the mechanistic basis for this defect in ERAD: the entrapment of the essential ERAD proteins Npl4, Ufd1, and p97 by polyQ-expanded htt fragments. polyglutamine 136-141 huntingtin Rattus norvegicus 151-154 19015277-7 2008 Our results establish that impaired ER protein homeostasis is a broad and highly conserved contributor to polyQ toxicity in yeast, in PC12 cells, and, importantly, in striatal cells expressing full-length polyQ-expanded huntingtin. polyglutamine 106-111 huntingtin Rattus norvegicus 220-230 19015277-7 2008 Our results establish that impaired ER protein homeostasis is a broad and highly conserved contributor to polyQ toxicity in yeast, in PC12 cells, and, importantly, in striatal cells expressing full-length polyQ-expanded huntingtin. polyglutamine 205-210 huntingtin Rattus norvegicus 220-230 19559011-1 2009 Huntington"s disease is a progressive neurodegenerative disorder caused by a polyglutamine expansion near the N-terminus of huntingtin. polyglutamine 77-90 huntingtin Rattus norvegicus 124-134 19559011-3 2009 We have studied gene expression profiles by short oligo array using an inducible PC12 cell model expressing an N-terminal huntingtin fragment with expanded polyglutamine (Htt-N63-148Q). polyglutamine 156-169 huntingtin Rattus norvegicus 122-132 19559011-3 2009 We have studied gene expression profiles by short oligo array using an inducible PC12 cell model expressing an N-terminal huntingtin fragment with expanded polyglutamine (Htt-N63-148Q). polyglutamine 156-169 huntingtin Rattus norvegicus 171-174 19247483-2 2009 In Huntington"s disease (HD), a disorder caused by an abnormal expansion of a polyglutamine tract in the protein huntingtin (Htt), the role of aging is unclear. polyglutamine 78-91 huntingtin Rattus norvegicus 113-123 19247483-2 2009 In Huntington"s disease (HD), a disorder caused by an abnormal expansion of a polyglutamine tract in the protein huntingtin (Htt), the role of aging is unclear. polyglutamine 78-91 huntingtin Rattus norvegicus 125-128 18753376-1 2008 Huntington"s disease (HD) is a polyglutamine [poly(Q)] disease with an expanded poly(Q) stretch in the N terminus of the huntingtin protein (htt). polyglutamine 31-44 huntingtin Rattus norvegicus 141-144 16087879-2 2005 We found that PC12 cells expressing polyglutamine-expanded huntingtin exon 1 accumulated less mutant protein and showed decreased cell death when treated with clioquinol. polyglutamine 36-49 huntingtin Rattus norvegicus 59-69 18255062-3 2008 We show here that the expression of N-terminal huntingtin proteins with expanded polyglutamine (polyQ) repeats causes cell death in neuronal PC6.3 cell that involves endoplasmic reticulum (ER) stress. polyglutamine 81-94 huntingtin Rattus norvegicus 47-57 18255062-3 2008 We show here that the expression of N-terminal huntingtin proteins with expanded polyglutamine (polyQ) repeats causes cell death in neuronal PC6.3 cell that involves endoplasmic reticulum (ER) stress. polyglutamine 96-101 huntingtin Rattus norvegicus 47-57 16641246-1 2006 Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin (htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington"s disease (HD) pathogenesis. polyglutamine 49-63 huntingtin Rattus norvegicus 78-88 16641246-1 2006 Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin (htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington"s disease (HD) pathogenesis. polyglutamine 49-63 huntingtin Rattus norvegicus 90-93 16641246-1 2006 Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin (htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington"s disease (HD) pathogenesis. polyglutamine 65-73 huntingtin Rattus norvegicus 78-88 16641246-1 2006 Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin (htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington"s disease (HD) pathogenesis. polyglutamine 65-73 huntingtin Rattus norvegicus 90-93 17905594-1 2008 Huntington"s disease (HD) results from an abnormal polyglutamine extension in the N-terminal region of the huntingtin protein. polyglutamine 51-64 huntingtin Rattus norvegicus 107-117 16452687-0 2006 Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421. polyglutamine 52-65 huntingtin Rattus norvegicus 66-76 16452687-0 2006 Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421. polyglutamine 52-65 huntingtin Rattus norvegicus 109-119 16452687-1 2006 Huntington"s disease (HD) is caused by an abnormal expanded polyglutamine (polyQ) repeat in the huntingtin protein. polyglutamine 60-73 huntingtin Rattus norvegicus 96-106 16452687-1 2006 Huntington"s disease (HD) is caused by an abnormal expanded polyglutamine (polyQ) repeat in the huntingtin protein. polyglutamine 75-80 huntingtin Rattus norvegicus 96-106 16452687-2 2006 Insulin-like growth factor-1 acting through the prosurvival kinase Akt mediates the phosphorylation of huntingtin at S421 and inhibits the toxicity of polyQ-expanded huntingtin in cell culture, suggesting that compounds enhancing phosphorylation are of therapeutic interest. polyglutamine 151-156 huntingtin Rattus norvegicus 166-176 16330479-1 2006 Huntington"s disease (HD) is a devastating neurodegenerative disorder caused by an expanded polyglutamine (polyQ) tract within the huntingtin protein (Htt). polyglutamine 92-105 huntingtin Rattus norvegicus 131-141 16330479-1 2006 Huntington"s disease (HD) is a devastating neurodegenerative disorder caused by an expanded polyglutamine (polyQ) tract within the huntingtin protein (Htt). polyglutamine 92-105 huntingtin Rattus norvegicus 151-154 16330479-1 2006 Huntington"s disease (HD) is a devastating neurodegenerative disorder caused by an expanded polyglutamine (polyQ) tract within the huntingtin protein (Htt). polyglutamine 107-112 huntingtin Rattus norvegicus 131-141 16330479-1 2006 Huntington"s disease (HD) is a devastating neurodegenerative disorder caused by an expanded polyglutamine (polyQ) tract within the huntingtin protein (Htt). polyglutamine 107-112 huntingtin Rattus norvegicus 151-154 15908226-2 2005 We previously developed an inducible PC12 cell model which expresses an N-terminal huntingtin fragment with an expanded poly Q repeat (N63-148Q) under the control of the tet-off system. polyglutamine 120-126 huntingtin Rattus norvegicus 83-93 15262266-2 2004 The assay measures the ability of drugs to protect cultured neuronal (PC12) cells from death caused by an expanded polyglutamine (poly Q) form of huntingtin exon 1. polyglutamine 115-128 huntingtin Rattus norvegicus 146-156 15689617-0 2005 cAMP-response element-binding protein contributes to suppression of the A2A adenosine receptor promoter by mutant Huntingtin with expanded polyglutamine residues. polyglutamine 139-152 huntingtin Rattus norvegicus 114-124 16033418-1 2005 Huntington"s disease (HD) is an autosomal dominantly inherited progressive neurodegenerative disorder caused by a CAG/polyglutamine repeat expansion in the gene encoding the huntingtin protein. polyglutamine 118-131 huntingtin Rattus norvegicus 174-184 15325591-3 2004 Two recent studies used microtubule disruption with nocodazole to inhibit aggresome formation and observed increased toxicity of expanded polyglutamines in the context of huntingtin exon 1 and a truncated androgen receptor. polyglutamine 138-152 huntingtin Rattus norvegicus 171-181 15262266-2 2004 The assay measures the ability of drugs to protect cultured neuronal (PC12) cells from death caused by an expanded polyglutamine (poly Q) form of huntingtin exon 1. polyglutamine 130-136 huntingtin Rattus norvegicus 146-156 11978824-0 2002 Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels, and protein length. polyglutamine 122-135 huntingtin Rattus norvegicus 39-49 12957494-1 2003 The gene defect in Huntington"s disease (HD) causes a polyglutamine expansion in the N-terminal region of huntingtin (N-htt). polyglutamine 54-67 huntingtin Rattus norvegicus 106-116 12957494-1 2003 The gene defect in Huntington"s disease (HD) causes a polyglutamine expansion in the N-terminal region of huntingtin (N-htt). polyglutamine 54-67 huntingtin Rattus norvegicus 120-123 12657886-1 2003 Huntington"s disease (HD) is an autosomal dominant neurodegenerative disorder caused by the abnormal expansion of a polyglutamine tract in the huntingtin protein. polyglutamine 116-129 huntingtin Rattus norvegicus 143-153 12657886-2 2003 We have developed PC12 cell lines in which the expression of an N-terminal truncation of huntingtin (N63) with either wild type (23Q) or expanded polyglutamine (148Q) can be induced by the removal of doxycycline. polyglutamine 146-159 huntingtin Rattus norvegicus 89-99 12952868-7 2003 In contrast, expression of a shorter htt form (htt171-82Q) did not require processing prior formation of nuclear aggregates and caused decrease of both DARPP-32 and NeuN neuronal markers at one month post-injection suggesting that polyQ pathology may be dependent on protein context. polyglutamine 231-236 huntingtin Rattus norvegicus 37-40 12799135-8 2003 Interestingly, using GST pull-down assays we found that a mutant Htt exon 1 construct with 53 polyglutamine (HD53Q) did not bind to NCoR in a polyglutamine-dependent fashion. polyglutamine 94-107 huntingtin Rattus norvegicus 65-68 12559971-3 2003 To examine whether these abnormalities might arise in part from direct effects of the expanded polyglutamine tract contained in mutant huntingtin, we have exposed normal rat liver and human lymphoblast mitochondria to glutathione S-transferase fusion proteins containing polyglutamine tracts of 0, 19, or 62 residues. polyglutamine 95-108 huntingtin Rattus norvegicus 135-145 12226089-2 2002 An in vitro model of polyglutamine-induced neuronal cell death was developed using truncated mutant huntingtin (Htt) and PC12 cells. polyglutamine 21-34 huntingtin Rattus norvegicus 100-110 12226089-2 2002 An in vitro model of polyglutamine-induced neuronal cell death was developed using truncated mutant huntingtin (Htt) and PC12 cells. polyglutamine 21-34 huntingtin Rattus norvegicus 112-115 12217956-2 2002 In order to model HD pathogenesis in a controlled system, we developed stable PC12 cell lines that express exon 1 fragments of the huntingtin gene with 23 or 74 polyglutamines driven by an inducible doxycycline (dox)-sensitive promoter (HD-23Q or HD-74Q). polyglutamine 161-175 huntingtin Rattus norvegicus 131-141 11733534-0 2002 Expression of full-length polyglutamine-expanded Huntingtin disrupts growth factor receptor signaling in rat pheochromocytoma (PC12) cells. polyglutamine 26-39 huntingtin Rattus norvegicus 49-59 11733534-5 2002 Expression of polyglutamine-expanded Huntingtin, but not normal Huntingtin, leads to a dramatic morphological change. polyglutamine 14-27 huntingtin Rattus norvegicus 37-47 11733534-9 2002 Our data demonstrate that polyglutamine-expanded Huntingtin disrupts cellular signaling mediated by both EGF and NGF receptors in PC12 cells. polyglutamine 26-39 huntingtin Rattus norvegicus 49-59 11733534-4 2002 To investigate the potential role of normal and polyglutamine-expanded Huntingtin in the regulation of growth factor receptor-mediated cellular signaling and biological function, we stably transfected full-length Huntingtin containing 16, 48, or 89 polyglutamine repeats into PC12 cells where cellular signaling mechanisms, mediated by nerve growth factor (NGF) or EGF receptors, are well characterized. polyglutamine 48-61 huntingtin Rattus norvegicus 71-81 9786889-0 1998 Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line. polyglutamine 14-27 huntingtin Rattus norvegicus 37-47 10964480-2 2000 It is caused by a polyglutamine repeat expansion in the huntingtin protein. polyglutamine 18-31 huntingtin Rattus norvegicus 56-66 9880591-1 1999 Recent evidence suggests that, in huntingtin and many other proteins, polyglutamine repeats are a toxic stimulus in neurodegenerative diseases. polyglutamine 70-83 huntingtin Rattus norvegicus 34-44 10377328-1 1999 Expanded polyglutamine tracts cause huntingtin and other proteins to accumulate and aggregate in neuronal nuclei. polyglutamine 9-22 huntingtin Rattus norvegicus 36-46 9786889-1 1998 Huntington"s disease is one of a growing number of hereditary neurodegenerative disorders caused by expansion of a polyglutamine stretch at the NH2 terminus of huntingtin. polyglutamine 115-128 huntingtin Rattus norvegicus 160-170 9786889-2 1998 To explore whether polyglutamine-expanded huntingtin induces neuronal toxicity, I examined the expression of the full-length of huntingtin with 16, 48, or 89 polyglutamine repeats in a rat hippocampal neuronal cell (HN33). polyglutamine 19-32 huntingtin Rattus norvegicus 42-52 9786889-3 1998 Expression of mutated huntingtin with 48 or 89 polyglutamine repeats stimulated c-Jun amino-terminal kinases (JNKs) activity and induced apoptotic cell death in HN33 cells while expression of normal huntingtin with 16 polyglutamine repeats had no toxic effect. polyglutamine 47-60 huntingtin Rattus norvegicus 22-32 9786889-3 1998 Expression of mutated huntingtin with 48 or 89 polyglutamine repeats stimulated c-Jun amino-terminal kinases (JNKs) activity and induced apoptotic cell death in HN33 cells while expression of normal huntingtin with 16 polyglutamine repeats had no toxic effect. polyglutamine 218-231 huntingtin Rattus norvegicus 22-32 9786889-5 1998 Taken together, my studies demonstrate that expression of polyglutamine-expanded huntingtin induces neuronal apoptosis via activation of the SEK1-JNK pathway. polyglutamine 58-71 huntingtin Rattus norvegicus 81-91 32975927-1 2020 ConspectusHuntington"s disease (HD) is a progressive, familial neurodegenerative disease triggered by the expansion of a polyglutamine (polyQ) track in the protein huntingtin (htt). polyglutamine 121-134 huntingtin Rattus norvegicus 164-174 33984771-1 2021 Aggregates of mutant huntingtin (mHTT) containing an expanded polyglutamine (polyQ) tract are hallmarks of Huntington"s Disease (HD). polyglutamine 62-75 huntingtin Rattus norvegicus 21-31 33984771-1 2021 Aggregates of mutant huntingtin (mHTT) containing an expanded polyglutamine (polyQ) tract are hallmarks of Huntington"s Disease (HD). polyglutamine 77-82 huntingtin Rattus norvegicus 21-31 9454836-1 1998 Huntingtin is the protein product of the gene for Huntington"s disease (HD) and carries a polyglutamine repeat that is expanded in HD (>36 units). polyglutamine 90-103 huntingtin Rattus norvegicus 0-10 9454836-2 1998 Huntingtin-associated protein (HAP1) is a neuronal protein and binds to huntingtin in association with the polyglutamine repeat. polyglutamine 107-120 huntingtin Rattus norvegicus 0-10 9454836-2 1998 Huntingtin-associated protein (HAP1) is a neuronal protein and binds to huntingtin in association with the polyglutamine repeat. polyglutamine 107-120 huntingtin Rattus norvegicus 72-82 32975927-1 2020 ConspectusHuntington"s disease (HD) is a progressive, familial neurodegenerative disease triggered by the expansion of a polyglutamine (polyQ) track in the protein huntingtin (htt). polyglutamine 121-134 huntingtin Rattus norvegicus 176-179 32975927-1 2020 ConspectusHuntington"s disease (HD) is a progressive, familial neurodegenerative disease triggered by the expansion of a polyglutamine (polyQ) track in the protein huntingtin (htt). polyglutamine 136-141 huntingtin Rattus norvegicus 164-174 32975927-1 2020 ConspectusHuntington"s disease (HD) is a progressive, familial neurodegenerative disease triggered by the expansion of a polyglutamine (polyQ) track in the protein huntingtin (htt). polyglutamine 136-141 huntingtin Rattus norvegicus 176-179 32975927-2 2020 PolyQ sequences up to Q36 in htt are not known to be toxic, while polyQ lengths above Q36 almost invariably lead to increased disease risk and decreased ages of onset. polyglutamine 0-5 huntingtin Rattus norvegicus 29-32 32675242-2 2020 It is caused by expansion of a cytosine-adenine-guanine triplet in the N-terminal domain of exon 1 in the huntingtin (HTT) gene that codes for an expanded polyglutamine stretch in the protein product which becomes aggregation prone. polyglutamine 155-168 huntingtin Rattus norvegicus 106-116 32574646-1 2020 Huntington disease (HD) is a neurodegenerative disorder caused by a polyglutamine expansion in the HTT gene. polyglutamine 68-81 huntingtin Rattus norvegicus 99-102 32675242-2 2020 It is caused by expansion of a cytosine-adenine-guanine triplet in the N-terminal domain of exon 1 in the huntingtin (HTT) gene that codes for an expanded polyglutamine stretch in the protein product which becomes aggregation prone. polyglutamine 155-168 huntingtin Rattus norvegicus 118-121 27984179-1 2017 Huntingtin-associated protein 1 (HAP1) is a neuronal interactor with causatively polyglutamine (polyQ)-expanded huntingtin in Huntington"s disease and also associated with pathologically polyQ-expanded androgen receptor (AR) in spinobulbar muscular atrophy (SBMA), being considered as a protective factor against neurodegenerative apoptosis. polyglutamine 81-94 huntingtin Rattus norvegicus 112-122 30916978-1 2019 Huntington"s disease (HD) is a neurodegenerative disease caused by polyglutamine expansion in the huntingtin protein. polyglutamine 67-80 huntingtin Rattus norvegicus 98-108 30171891-3 2018 Here we describe an engineered htt exon1 analog featuring a short polyQ sequence that nonetheless quickly forms amyloid fibrils and causes HD-like toxicity in rat neurons and Drosophila. polyglutamine 66-71 huntingtin Rattus norvegicus 31-34 28499347-2 2017 Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. polyglutamine 73-86 huntingtin Rattus norvegicus 31-41 28499347-2 2017 Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. polyglutamine 73-86 huntingtin Rattus norvegicus 43-46 28499347-2 2017 Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. polyglutamine 73-86 huntingtin Rattus norvegicus 128-131 28499347-2 2017 Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. polyglutamine 88-93 huntingtin Rattus norvegicus 31-41 28499347-2 2017 Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. polyglutamine 88-93 huntingtin Rattus norvegicus 43-46 28499347-2 2017 Proteolytic cleavage of mutant huntingtin (Htt) protein with an expanded polyglutamine (polyQ) stretch results in production of Htt fragments that aggregate and induce impaired ubiquitin proteasome, mitochondrial functioning and transcriptional dysregulation. polyglutamine 88-93 huntingtin Rattus norvegicus 128-131 27984179-1 2017 Huntingtin-associated protein 1 (HAP1) is a neuronal interactor with causatively polyglutamine (polyQ)-expanded huntingtin in Huntington"s disease and also associated with pathologically polyQ-expanded androgen receptor (AR) in spinobulbar muscular atrophy (SBMA), being considered as a protective factor against neurodegenerative apoptosis. polyglutamine 96-101 huntingtin Rattus norvegicus 112-122 27717053-1 2017 Huntington"s disease, an inherited neurodegenerative disorder, results from abnormal polyglutamine extension in the N-terminal region of the huntingtin protein. polyglutamine 85-98 huntingtin Rattus norvegicus 141-151 23392662-3 2013 Wild-type MKP-1 overexpression inhibited apoptosis in primary striatal neurons exposed to an N-terminal fragment of polyglutamine-expanded huntingtin (Htt171-82Q), blocking caspase-3 activation and significantly reducing neuronal cell death. polyglutamine 116-129 huntingtin Rattus norvegicus 139-149 25744490-1 2015 Huntingtin-associated protein 1 (HAP1) is enriched in neurons and binds to polyglutamine-expanded huntingtin. polyglutamine 75-88 huntingtin Rattus norvegicus 98-108 24352657-2 2014 We have used an established cell-based assay employing a PC12 cell line overexpressing truncated exon 1 of Htt with a 103-residue polyQ expansion that yields polyQ-Htt aggregates to investigate the fate of polyQ-Htt-drug complexes. polyglutamine 158-163 huntingtin Rattus norvegicus 164-167 24352657-2 2014 We have used an established cell-based assay employing a PC12 cell line overexpressing truncated exon 1 of Htt with a 103-residue polyQ expansion that yields polyQ-Htt aggregates to investigate the fate of polyQ-Htt-drug complexes. polyglutamine 158-163 huntingtin Rattus norvegicus 164-167 24352657-4 2014 In light of these properties, we investigated the effect of scyllo-inositol on polyQ-Htt accumulation. polyglutamine 79-84 huntingtin Rattus norvegicus 85-88 23720495-1 2013 Huntington"s disease (HD) is a neurodegenerative disorder caused by an abnormal expansion of a CAG repeat encoding a polyglutamine tract in the huntingtin (Htt) protein. polyglutamine 117-130 huntingtin Rattus norvegicus 144-154 23720495-1 2013 Huntington"s disease (HD) is a neurodegenerative disorder caused by an abnormal expansion of a CAG repeat encoding a polyglutamine tract in the huntingtin (Htt) protein. polyglutamine 117-130 huntingtin Rattus norvegicus 156-159 23799534-1 2013 Huntington"s disease (HD) is a devastating neurodegenerative disorder caused by abnormal polyglutamine expansion in the huntingtin protein (Exp-Htt). polyglutamine 89-102 huntingtin Rattus norvegicus 120-130 27751235-0 2016 Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract. polyglutamine 109-114 huntingtin Rattus norvegicus 74-84 27751235-2 2016 In Huntington"s disease (HD), neurotoxicity correlates with an increased aggregation propensity of a polyglutamine (polyQ) expansion in exon 1 of mutant huntingtin protein (mHtt). polyglutamine 101-114 huntingtin Rattus norvegicus 153-163 27751235-2 2016 In Huntington"s disease (HD), neurotoxicity correlates with an increased aggregation propensity of a polyglutamine (polyQ) expansion in exon 1 of mutant huntingtin protein (mHtt). polyglutamine 116-121 huntingtin Rattus norvegicus 153-163 27271685-1 2016 Expansion of the polyglutamine (polyQ) track of the Huntingtin (HTT) protein above 36 is associated with a sharply enhanced risk of Huntington"s disease (HD). polyglutamine 17-30 huntingtin Rattus norvegicus 52-62 27271685-1 2016 Expansion of the polyglutamine (polyQ) track of the Huntingtin (HTT) protein above 36 is associated with a sharply enhanced risk of Huntington"s disease (HD). polyglutamine 17-30 huntingtin Rattus norvegicus 64-67 27271685-1 2016 Expansion of the polyglutamine (polyQ) track of the Huntingtin (HTT) protein above 36 is associated with a sharply enhanced risk of Huntington"s disease (HD). polyglutamine 32-37 huntingtin Rattus norvegicus 52-62 27271685-1 2016 Expansion of the polyglutamine (polyQ) track of the Huntingtin (HTT) protein above 36 is associated with a sharply enhanced risk of Huntington"s disease (HD). polyglutamine 32-37 huntingtin Rattus norvegicus 64-67 27271685-3 2016 One hypothesis is that polyQ expansion induces an alternative, toxic conformation in the HTT exon1 monomer. polyglutamine 23-28 huntingtin Rattus norvegicus 89-92 27271685-7 2016 We find that, in vitro, mutant HTT exon1 peptides engage in polyQ repeat length dependent dimer and tetramer formation, followed by time dependent formation of diffusible spherical and fibrillar oligomers and finally by larger, sedimentable amyloid fibrils. polyglutamine 60-65 huntingtin Rattus norvegicus 31-34 23392662-5 2013 Overexpression of MKP-1 prevented the polyglutamine-expanded huntingtin-induced activation of c-Jun N-terminal kinases (JNKs) and p38 MAPKs, whereas extracellular signal-regulated kinase (ERK) 1/2 activation was not altered by either polyglutamine-expanded Htt or MKP-1. polyglutamine 38-51 huntingtin Rattus norvegicus 61-71 23392662-5 2013 Overexpression of MKP-1 prevented the polyglutamine-expanded huntingtin-induced activation of c-Jun N-terminal kinases (JNKs) and p38 MAPKs, whereas extracellular signal-regulated kinase (ERK) 1/2 activation was not altered by either polyglutamine-expanded Htt or MKP-1. polyglutamine 38-51 huntingtin Rattus norvegicus 257-260 23392662-5 2013 Overexpression of MKP-1 prevented the polyglutamine-expanded huntingtin-induced activation of c-Jun N-terminal kinases (JNKs) and p38 MAPKs, whereas extracellular signal-regulated kinase (ERK) 1/2 activation was not altered by either polyglutamine-expanded Htt or MKP-1. polyglutamine 234-247 huntingtin Rattus norvegicus 61-71 21079601-6 2010 Expression of polyglutamine-expanded huntingtin exon 1 in PC12 cells caused PDI to accumulate at mitochondrial-associated ER membranes and trigger apoptotic cell death via mitochondrial outer-membrane permeabilization. polyglutamine 14-27 huntingtin Rattus norvegicus 37-47 21452052-1 2011 Huntington"s disease (HD) is caused by an abnormal expansion of CAG trinucleotide repeats encoding polyglutamine (polyQ) in the first exon of the huntingtin (htt) gene. polyglutamine 99-112 huntingtin Rattus norvegicus 146-156 21452052-1 2011 Huntington"s disease (HD) is caused by an abnormal expansion of CAG trinucleotide repeats encoding polyglutamine (polyQ) in the first exon of the huntingtin (htt) gene. polyglutamine 99-112 huntingtin Rattus norvegicus 158-161 21452052-1 2011 Huntington"s disease (HD) is caused by an abnormal expansion of CAG trinucleotide repeats encoding polyglutamine (polyQ) in the first exon of the huntingtin (htt) gene. polyglutamine 114-119 huntingtin Rattus norvegicus 146-156 21452052-1 2011 Huntington"s disease (HD) is caused by an abnormal expansion of CAG trinucleotide repeats encoding polyglutamine (polyQ) in the first exon of the huntingtin (htt) gene. polyglutamine 114-119 huntingtin Rattus norvegicus 158-161 22410294-1 2012 Huntington"s disease (HD) is an inheritable neurological disorder caused by an abnormal expansion of the polyglutamine tract in the N-terminus of the protein huntingtin (htt). polyglutamine 105-118 huntingtin Rattus norvegicus 158-168 22410294-1 2012 Huntington"s disease (HD) is an inheritable neurological disorder caused by an abnormal expansion of the polyglutamine tract in the N-terminus of the protein huntingtin (htt). polyglutamine 105-118 huntingtin Rattus norvegicus 170-173