PMID-sentid	Pub_year	Sent_text	compound_name	comp_offset	prot_official_name	organism	prot_offset
18951640-4	2008	Here, we highlight how mutant huntingtin (mtHtt) might cause mitochondrial dysfunction by either perturbing transcription of nuclear-encoded mitochondrial proteins or by direct interaction with the organelle and modulation of respiration, mitochondrial membrane potential and Ca(2+) buffering.	mthtt	42-47	huntingtin	Homo sapiens	30-40
27913212-10	2017	These results indicate that the OMM protein MCL1 is degraded by the VCP-UBXD1 complex and that the process is promoted by the presence of mtHtt.	mthtt	138-143	UBX domain protein 6	Homo sapiens	72-77
29066943-2	2017	We have previously demonstrated that spliceosome-mediated trans-splicing is a viable molecular strategy to specifically reduce and repair mutant HTT (mtHTT).	mthtt	150-155	huntingtin	Homo sapiens	145-148
23966247-7	2013	CONCLUSIONS: The HTRF assay can effectively measure mtHtt in multicenter sample sets and may be useful in trials of therapies targeting huntingtin.	mthtt	52-57	huntingtin	Homo sapiens	136-146
27561680-3	2016	Here we show that VCP is selectively translocated to the mitochondria, where it is bound to mtHtt in various HD models.	mthtt	92-97	valosin containing protein	Mus musculus	18-21
24121117-3	2014	Here, we use the cre-lox system to drive expression of mutant huntingtin (mthtt) in parvalbumin (PV)-positive neurons and find that mutant mice exhibit diffuse mthtt immunoreactivity in PV-rich areas at 10months of age and mthtt aggregates in PV-positive processes at 24months of age.	mthtt	74-79	huntingtin	Mus musculus	62-72
24121117-3	2014	Here, we use the cre-lox system to drive expression of mutant huntingtin (mthtt) in parvalbumin (PV)-positive neurons and find that mutant mice exhibit diffuse mthtt immunoreactivity in PV-rich areas at 10months of age and mthtt aggregates in PV-positive processes at 24months of age.	mthtt	74-79	parvalbumin	Mus musculus	84-95
24121117-3	2014	Here, we use the cre-lox system to drive expression of mutant huntingtin (mthtt) in parvalbumin (PV)-positive neurons and find that mutant mice exhibit diffuse mthtt immunoreactivity in PV-rich areas at 10months of age and mthtt aggregates in PV-positive processes at 24months of age.	mthtt	160-165	huntingtin	Mus musculus	62-72
24121117-3	2014	Here, we use the cre-lox system to drive expression of mutant huntingtin (mthtt) in parvalbumin (PV)-positive neurons and find that mutant mice exhibit diffuse mthtt immunoreactivity in PV-rich areas at 10months of age and mthtt aggregates in PV-positive processes at 24months of age.	mthtt	160-165	huntingtin	Mus musculus	62-72
24231356-9	2013	Furthermore, P110-TAT treatment suppressed mtHtt-induced association of p53 with mitochondria in multiple HD models.	mthtt	43-48	tumor protein p53	Homo sapiens	72-75
26637326-5	2016	Ectopic expression of ENC1 alone induces the accumulation of detergent-resistant mtHTT aggregates and downregulation of ENC1 alleviates ER stress-induced mtHTT aggregation.	mthtt	81-86	ectodermal-neural cortex 1	Homo sapiens	22-26
26637326-5	2016	Ectopic expression of ENC1 alone induces the accumulation of detergent-resistant mtHTT aggregates and downregulation of ENC1 alleviates ER stress-induced mtHTT aggregation.	mthtt	154-159	ectodermal-neural cortex 1	Homo sapiens	22-26
26637326-5	2016	Ectopic expression of ENC1 alone induces the accumulation of detergent-resistant mtHTT aggregates and downregulation of ENC1 alleviates ER stress-induced mtHTT aggregation.	mthtt	154-159	ectodermal-neural cortex 1	Homo sapiens	120-124
26637326-9	2016	Interestingly, ENC1 colocalizes with mtHTT aggregates and its C-terminal Kelch domain is required for interfering with the access of p62 to ubiquitinated mtHTT aggregates, thus inhibiting cargo recognition of p62.	mthtt	37-42	ectodermal-neural cortex 1	Homo sapiens	15-19
26637326-9	2016	Interestingly, ENC1 colocalizes with mtHTT aggregates and its C-terminal Kelch domain is required for interfering with the access of p62 to ubiquitinated mtHTT aggregates, thus inhibiting cargo recognition of p62.	mthtt	154-159	ectodermal-neural cortex 1	Homo sapiens	15-19
26637326-9	2016	Interestingly, ENC1 colocalizes with mtHTT aggregates and its C-terminal Kelch domain is required for interfering with the access of p62 to ubiquitinated mtHTT aggregates, thus inhibiting cargo recognition of p62.	mthtt	154-159	nucleoporin 62	Homo sapiens	133-136
26637326-10	2016	Accordingly, knockdown of ENC1 expression enhances colocalization of p62 with mtHTT aggregates.	mthtt	78-83	ectodermal-neural cortex 1	Homo sapiens	26-30
26637326-10	2016	Accordingly, knockdown of ENC1 expression enhances colocalization of p62 with mtHTT aggregates.	mthtt	78-83	nucleoporin 62	Homo sapiens	69-72
26637326-11	2016	Consequently, ENC1 knockdown relieves death of neuronal cells expressing mtHTT under ER stress.	mthtt	73-78	ectodermal-neural cortex 1	Homo sapiens	14-18
26637326-12	2016	These results suggest that ENC1 interacts with the phosphorylated p62 to impair autophagic degradation of mtHTT aggregates and affects cargo recognition failure under ER stress, leading to the accumulation and neurotoxicity of mtHTT aggregates.	mthtt	106-111	ectodermal-neural cortex 1	Homo sapiens	27-31
26637326-12	2016	These results suggest that ENC1 interacts with the phosphorylated p62 to impair autophagic degradation of mtHTT aggregates and affects cargo recognition failure under ER stress, leading to the accumulation and neurotoxicity of mtHTT aggregates.	mthtt	106-111	nucleoporin 62	Homo sapiens	66-69
26637326-12	2016	These results suggest that ENC1 interacts with the phosphorylated p62 to impair autophagic degradation of mtHTT aggregates and affects cargo recognition failure under ER stress, leading to the accumulation and neurotoxicity of mtHTT aggregates.	mthtt	227-232	ectodermal-neural cortex 1	Homo sapiens	27-31
26637326-12	2016	These results suggest that ENC1 interacts with the phosphorylated p62 to impair autophagic degradation of mtHTT aggregates and affects cargo recognition failure under ER stress, leading to the accumulation and neurotoxicity of mtHTT aggregates.	mthtt	227-232	nucleoporin 62	Homo sapiens	66-69
22722716-10	2012	DMSO reduced not only soluble but also insoluble mtHTT (mutant huntingtin aggregates) expressions, which were masked in the presence of autophagy inhibitor.	mthtt	49-54	huntingtin	Homo sapiens	63-73
22593067-9	2012	Moreover, exogenous expression of PGC-1alpha reversed mtHtt-mediated increases in NMDAR(EX) activity and protected neurons against excitotoxic cell death.	mthtt	54-59	PPARG coactivator 1 alpha	Rattus norvegicus	34-44
22240898-5	2012	Knockdown of ATRX/dXNP improved the hatch rate of fly embryos expressing mtHtt (Q127).	mthtt	73-78	XNP	Drosophila melanogaster	13-17
22240898-5	2012	Knockdown of ATRX/dXNP improved the hatch rate of fly embryos expressing mtHtt (Q127).	mthtt	73-78	XNP	Drosophila melanogaster	18-22
22240898-6	2012	ATRX/dXNP overexpression exacerbated eye degeneration of eye-specific mtHtt (Q127) expressing flies.	mthtt	70-75	XNP	Drosophila melanogaster	0-4
22240898-6	2012	ATRX/dXNP overexpression exacerbated eye degeneration of eye-specific mtHtt (Q127) expressing flies.	mthtt	70-75	XNP	Drosophila melanogaster	5-9
22240898-7	2012	Our findings suggest that transcriptional alteration of ATRX by mtHtt is involved in pericentromeric heterochromatin condensation and contributes to the pathogenesis of HD.	mthtt	64-69	XNP	Drosophila melanogaster	56-60
22415443-6	2012	3-nitropropionic acid, on the aggregation of mutant huntingtin (mthtt) protein, whose misfolding and aggregation results in cellular abnormalities characteristic of HD.	mthtt	64-69	huntingtin	Homo sapiens	52-62
21278900-1	2010	A means for measuring levels of soluble huntingtin proteins in clinical samples is essential for assessing the biological effects of potential mutant huntingtin (mtHtt) modifying treatments being developed for Huntington"s disease (HD).	mthtt	162-167	huntingtin	Homo sapiens	40-50
21954231-5	2012	Ectopic expression of IRE1 led to its self-activation and accumulated detergent-resistant mtHTT aggregates.	mthtt	90-95	endoplasmic reticulum (ER) to nucleus signalling 2	Mus musculus	22-26
21954231-6	2012	Treatment of neuronal cells with ER stress insults, tunicamycin and thapsigargin, increased mtHTT aggregation via IRE1 activation.	mthtt	92-97	endoplasmic reticulum (ER) to nucleus signalling 2	Mus musculus	114-118
21954231-7	2012	The kinase activity of IRE1, but not the endoribonuclease activity, was necessary to stimulate mtHTT aggregation and increased death of neuronal cells, including SH-SY5Y and STHdhQ111/111 huntingtin knock-in striatal cells.	mthtt	95-100	endoplasmic reticulum (ER) to nucleus signalling 2	Mus musculus	23-27
21954231-8	2012	Interestingly, ER stress impaired autophagy flux via IRE1-TRAF2 pathway, thus enhancing cellular accumulation of mtHTT.	mthtt	113-118	endoplasmic reticulum (ER) to nucleus signalling 2	Mus musculus	53-57
21954231-8	2012	Interestingly, ER stress impaired autophagy flux via IRE1-TRAF2 pathway, thus enhancing cellular accumulation of mtHTT.	mthtt	113-118	TNF receptor-associated factor 2	Mus musculus	58-63
21954231-9	2012	Atg5 deficiency in M5-7 cells increased mtHTT aggregation but blocked ER stress-induced mtHTT aggregation.	mthtt	40-45	autophagy related 5	Mus musculus	0-4
21954231-9	2012	Atg5 deficiency in M5-7 cells increased mtHTT aggregation but blocked ER stress-induced mtHTT aggregation.	mthtt	88-93	autophagy related 5	Mus musculus	0-4
21954231-11	2012	Moreover, down-regulation of IRE1 expression rescues the rough-eye phenotype by mtHTT in a HD fly model.	mthtt	80-85	Inositol-requiring enzyme-1	Drosophila melanogaster	29-33
21954231-12	2012	These results suggest that IRE1 plays an essential role in ER stress-mediated aggregation of mtHTT via the inhibition of autophagy flux and thus neuronal toxicity of mtHTT aggregates in HD.	mthtt	93-98	Inositol-requiring enzyme-1	Drosophila melanogaster	27-31
21954231-12	2012	These results suggest that IRE1 plays an essential role in ER stress-mediated aggregation of mtHTT via the inhibition of autophagy flux and thus neuronal toxicity of mtHTT aggregates in HD.	mthtt	166-171	Inositol-requiring enzyme-1	Drosophila melanogaster	27-31
21278900-1	2010	A means for measuring levels of soluble huntingtin proteins in clinical samples is essential for assessing the biological effects of potential mutant huntingtin (mtHtt) modifying treatments being developed for Huntington"s disease (HD).	mthtt	162-167	huntingtin	Homo sapiens	150-160
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	79-84	glutamate receptor, ionotropic, NMDA1 (zeta 1)	Mus musculus	9-53
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	79-84	glutamate receptor, ionotropic, NMDA1 (zeta 1)	Mus musculus	55-60
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	199-204	t-complex protein 1	Mus musculus	102-113
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	79-84	t-complex protein 1	Mus musculus	102-113
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	199-204	t-complex protein 1	Mus musculus	115-120
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	79-84	t-complex protein 1	Mus musculus	115-120
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	80-85	cAMP responsive element binding protein 1	Mus musculus	152-195
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	80-85	cAMP responsive element binding protein 1	Mus musculus	197-201
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	199-204	glutamate receptor, ionotropic, NMDA1 (zeta 1)	Mus musculus	9-53
19915593-4	2009	Synaptic N-methyl-D-aspartate-type glutamate receptor (NMDAR) activity induces mtHtt inclusions via a T complex-1 (TCP-1) ring complex (TRiC)-dependent mechanism, rendering neurons more resistant to mtHtt-mediated cell death.	mthtt	199-204	glutamate receptor, ionotropic, NMDA1 (zeta 1)	Mus musculus	55-60
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	80-85	peroxisome proliferative activated receptor, gamma, coactivator 1 alpha	Mus musculus	203-270
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	80-85	peroxisome proliferative activated receptor, gamma, coactivator 1 alpha	Mus musculus	272-282
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	80-85	RASD family, member 2	Mus musculus	365-369
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	416-421	cAMP responsive element binding protein 1	Mus musculus	152-195
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	416-421	cAMP responsive element binding protein 1	Mus musculus	197-201
19915593-5	2009	In contrast, stimulation of extrasynaptic NMDARs increases the vulnerability of mtHtt-containing neurons to cell death by impairing the neuroprotective cyclic AMP response element-binding protein (CREB)-peroxisome proliferator-activated receptor-gamma coactivator-1alpha (PGC-1alpha) cascade and increasing the level of the small guanine nucleotide-binding protein Rhes, which is known to sumoylate and disaggregate mtHtt.	mthtt	416-421	peroxisome proliferative activated receptor, gamma, coactivator 1 alpha	Mus musculus	203-270
19240033-3	2009	Ectopic expression of SCAMP5 augments the formation of ubiquitin-positive and detergent-resistant aggregates of mutant huntingtin (mtHTT).	mthtt	131-136	secretory carrier membrane protein 5	Mus musculus	22-28
19240033-3	2009	Ectopic expression of SCAMP5 augments the formation of ubiquitin-positive and detergent-resistant aggregates of mutant huntingtin (mtHTT).	mthtt	131-136	huntingtin	Mus musculus	119-129
19240033-4	2009	Expression of SCAMP5 is markedly increased in the striatum of Huntington disease patients and is induced in cultured striatal neurons by endoplasmic reticulum (ER) stress or by mtHTT.	mthtt	177-182	secretory carrier membrane protein 5	Homo sapiens	14-20
19240033-5	2009	The increase of SCAMP5 impairs endocytosis, which in turn enhances mtHTT aggregation.	mthtt	67-72	secretory carrier membrane protein 5	Mus musculus	16-22
19240033-6	2009	On the contrary, down-regulation of SCAMP5 alleviates ER stress-induced mtHTT aggregation and endocytosis inhibition.	mthtt	72-77	secretory carrier membrane protein 5	Mus musculus	36-42
19240033-8	2009	Taken together, these results suggest that exposure to ER stress increases SCAMP5 in the striatum, which positively regulates mtHTT aggregation via the endocytosis pathway.	mthtt	126-131	secretory carrier membrane protein 5	Mus musculus	75-81