| scholarly article | Q13442814 |
| P50 | author | Elena Cattaneo | Q14754989 |
| P2093 | author name string | A H Sharp | |
| D Rigamonti | |||
| M E MacDonald | |||
| F Trettel | |||
| F Persichetti | |||
| V C Wheeler | |||
| P Hilditch-Maguire | |||
| P4510 | describes a project that uses | STHdhQ111 | Q54970579 |
| P433 | issue | 19 | |
| P921 | main subject | phenotype | Q104053 |
| striatum | Q1319792 | ||
| cell line | Q21014462 | ||
| P304 | page(s) | 2799-2809 | |
| P577 | publication date | 2000-11-01 | |
| P1433 | published in | Human Molecular Genetics | Q2720965 |
| P1476 | title | Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells | |
| P478 | volume | 9 |
| Q50206457 | 7,8-dihydroxyflavone ameliorates cognitive and motor deficits in a Huntington's disease mouse model through specific activation of the PLCγ1 pathway |
| Q92860328 | A Multi-Omics Interpretable Machine Learning Model Reveals Modes of Action of Small Molecules |
| Q37528334 | A failure in energy metabolism and antioxidant uptake precede symptoms of Huntington's disease in mice. |
| Q28485362 | A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease |
| Q54963199 | A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease. |
| Q27301289 | A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease. |
| Q37622579 | A large scale Huntingtin protein interaction network implicates Rho GTPase signaling pathways in Huntington disease |
| Q57299362 | A newly generated neuronal cell model of CLN7 disease reveals aberrant lysosome motility and impaired cell survival |
| Q33543359 | A novel approach to investigate tissue-specific trinucleotide repeat instability |
| Q28389607 | A novel manganese-dependent ATM-p53 signaling pathway is selectively impaired in patient-based neuroprogenitor and murine striatal models of Huntington's disease |
| Q93196016 | A novel specific PERK activator reduces toxicity and extends survival in Huntington's disease models |
| Q64882921 | A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths. |
| Q47236927 | A peptidylic inhibitor for neutralizing expanded CAG RNA-induced nucleolar stress in polyglutamine diseases |
| Q28474016 | A role for oxidized DNA precursors in Huntington's disease-like striatal neurodegeneration |
| Q33920945 | A selective inhibitor of histone deacetylase 3 prevents cognitive deficits and suppresses striatal CAG repeat expansions in Huntington's disease mice |
| Q35214858 | A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity |
| Q36608838 | AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington's disease. |
| Q64120447 | ATAD3A oligomerization causes neurodegeneration by coupling mitochondrial fragmentation and bioenergetics defects |
| Q36028454 | ATRX induction by mutant huntingtin via Cdx2 modulates heterochromatin condensation and pathology in Huntington's disease |
| Q47288108 | Aberrant subcellular localization of SQSTM1/p62 contributes to increased vulnerability to proteotoxic stress recovery in Huntington's disease |
| Q42067930 | Activating transcription factor 6 derepression mediates neuroprotection in Huntington disease. |
| Q45288978 | Adenovirus vector-based in vitro neuronal cell model for Huntington's disease with human disease-like differential aggregation and degeneration |
| Q91013759 | Allele-selective lowering of mutant HTT protein by HTT-LC3 linker compounds |
| Q91530448 | Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington's disease |
| Q45295287 | Allele-specific regulation of mutant Huntingtin by Wig1, a downstream target of p53. |
| Q48715164 | Altered Expression of the Long Noncoding RNA NEAT1 in Huntington's Disease. |
| Q57466010 | Altered Levels of Long NcRNAs Meg3 and Neat1 in Cell and Animal models of Huntington's Disease |
| Q37352446 | Altered histone monoubiquitylation mediated by mutant huntingtin induces transcriptional dysregulation |
| Q64931945 | Altered lactate metabolism in Huntington's disease is dependent on GLUT3 expression. |
| Q35912172 | Altered lysosomal positioning affects lysosomal functions in a cellular model of Huntington's disease |
| Q34071987 | Altered manganese homeostasis and manganese toxicity in a Huntington's disease striatal cell model are not explained by defects in the iron transport system. |
| Q50230269 | Antagonism of Dopamine Receptor 2 Long Affects Cannabinoid Receptor 1 Signaling in a Cell Culture Model of Striatal Medium Spiny Projection Neurons |
| Q44962020 | Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy |
| Q43284919 | BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution |
| Q45289973 | Bcl-2/adenovirus E1B 19-kDa interacting protein (BNip3) has a key role in the mitochondrial dysfunction induced by mutant huntingtin. |
| Q36824396 | Beyond the redox imbalance: Oxidative stress contributes to an impaired GLUT3 modulation in Huntington's disease |
| Q38809361 | Biased Type 1 Cannabinoid Receptor Signaling Influences Neuronal Viability in a Cell Culture Model of Huntington Disease |
| Q49077190 | Biologic models of neurodegenerative disorders. |
| Q45300857 | CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity. |
| Q36615488 | CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington's disease. |
| Q40020558 | Calcineurin is involved in the early activation of NMDA-mediated cell death in mutant huntingtin knock-in striatal cells |
| Q35906784 | Calcium Handling by Endoplasmic Reticulum and Mitochondria in a Cell Model of Huntington's Disease. |
| Q40031554 | Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease |
| Q38848638 | Cannabidiol is a negative allosteric modulator of the cannabinoid CB1 receptor |
| Q33814719 | Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease |
| Q39546142 | Cellular fura-2 manganese extraction assay (CFMEA). |
| Q35227496 | Changes in BiP availability reveal hypersensitivity to acute endoplasmic reticulum stress in cells expressing mutant huntingtin |
| Q35098204 | Characterization of mouse striatal precursor cell lines expressing functional dopamine receptors |
| Q30787791 | Cholesterol Modifies Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes |
| Q24292909 | Ciliogenesis is regulated by a huntingtin-HAP1-PCM1 pathway and is altered in Huntington disease |
| Q47564614 | Connecting Neuronal Cell Protective Pathways and Drug Combinations in a Huntington's Disease Model through the Application of Quantitative Systems Pharmacology. |
| Q24548047 | Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase |
| Q21560855 | Cytosolic guanine nucledotide binding deficient form of transglutaminase 2 (R580a) potentiates cell death in oxygen glucose deprivation |
| Q40042333 | Cytotoxicity is predicted by unbound and not total bilirubin concentration. |
| Q44420154 | DNA vaccination against mutant huntingtin ameliorates the HDR6/2 diabetic phenotype. |
| Q26822846 | Defining the role of the Bcl-2 family proteins in Huntington's disease |
| Q28565331 | Delivery of GABAARs to synapses is mediated by HAP1-KIF5 and disrupted by mutant huntingtin. |
| Q36274811 | Deregulation of BRCA1 leads to impaired spatiotemporal dynamics of γ-H2AX and DNA damage responses in Huntington's disease |
| Q35782283 | Disease Modifying Potential of Glatiramer Acetate in Huntington's Disease |
| Q39785057 | Disease-toxicant screen reveals a neuroprotective interaction between Huntington's disease and manganese exposure |
| Q36080317 | Dithiol-based compounds maintain expression of antioxidant protein peroxiredoxin 1 that counteracts toxicity of mutant huntingtin |
| Q37727587 | Dopamine D2 receptor relies upon PPM/PP2C protein phosphatases to dephosphorylate huntingtin protein |
| Q39933436 | Dopaminergic and glutamatergic signaling crosstalk in Huntington's disease neurodegeneration: the role of p25/cyclin-dependent kinase 5. |
| Q48514212 | Drp1 phosphorylation by MAPK1 causes mitochondrial dysfunction in cell culture model of Huntington's disease |
| Q64269415 | Dual Role of Ribosome-Binding Domain of NAC as a Potent Suppressor of Protein Aggregation and Aging-Related Proteinopathies |
| Q45290680 | Dysregulation of REST-regulated coding and non-coding RNAs in a cellular model of Huntington's disease |
| Q34293784 | Dysregulation of system xc(-) expression induced by mutant huntingtin in a striatal neuronal cell line and in R6/2 mice |
| Q34026790 | Dysregulation of upstream binding factor-1 acetylation at K352 is linked to impaired ribosomal DNA transcription in Huntington's disease |
| Q53253751 | ENC1 Modulates the Aggregation and Neurotoxicity of Mutant Huntingtin Through p62 Under ER Stress. |
| Q35110977 | ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin |
| Q37574135 | ESET methylates UBF at K232/254 and regulates nucleolar heterochromatin plasticity and rDNA transcription. |
| Q37580232 | Early defect of transforming growth factor β1 formation in Huntington's disease. |
| Q44605170 | Enhanced Akt signaling is an early pro-survival response that reflects N-methyl-D-aspartate receptor activation in Huntington's disease knock-in striatal cells |
| Q30539113 | Epigenetic regulation of cholinergic receptor M1 (CHRM1) by histone H3K9me3 impairs Ca(2+) signaling in Huntington's disease |
| Q50437564 | Epigenetics of Huntington's Disease |
| Q30536567 | Exogenous delivery of chaperonin subunit fragment ApiCCT1 modulates mutant Huntingtin cellular phenotypes |
| Q36637306 | Expression of RNAs Coding for Metal Transporters in Blood of Patients with Huntington's Disease |
| Q45304727 | Expression of the Huntington's disease transgene in neural stem cell cultures from R6/2 transgenic mice |
| Q36598186 | Extensive changes in DNA methylation are associated with expression of mutant huntingtin. |
| Q34212651 | FGF-2 promotes neurogenesis and neuroprotection and prolongs survival in a transgenic mouse model of Huntington's disease |
| Q41873152 | Forkhead box protein p1 is a transcriptional repressor of immune signaling in the CNS: implications for transcriptional dysregulation in Huntington disease |
| Q33755845 | Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression |
| Q28507781 | Functional roles for the striatal-enriched transcription factor, Bcl11b, in the control of striatal gene expression and transcriptional dysregulation in Huntington's disease |
| Q37574419 | Gene therapy for neurodegenerative diseases based on lentiviral vectors |
| Q34017754 | Genome-wide identification of Bcl11b gene targets reveals role in brain-derived neurotrophic factor signaling |
| Q35959686 | Genomic Instability Associated with p53 Knockdown in the Generation of Huntington's Disease Human Induced Pluripotent Stem Cells |
| Q39178944 | Glutamate transporter expression and function in a striatal neuronal model of Huntington's disease |
| Q37241010 | Golgi protein ACBD3 mediates neurotoxicity associated with Huntington's disease |
| Q35014895 | Grb2 is regulated by foxd3 and has roles in preventing accumulation and aggregation of mutant huntingtin |
| Q37612752 | HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response. |
| Q35585297 | HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity |
| Q34926539 | HDAC inhibition imparts beneficial transgenerational effects in Huntington's disease mice via altered DNA and histone methylation |
| Q33649916 | HIF prolyl hydroxylase inhibitors prevent neuronal death induced by mitochondrial toxins: therapeutic implications for Huntington's disease and Alzheimer's disease. |
| Q28386493 | HdhQ111 Mice Exhibit Tissue Specific Metabolite Profiles that Include Striatal Lipid Accumulation |
| Q90580774 | Hippo Signaling Pathway Dysregulation in Human Huntington's Disease Brain and Neuronal Stem Cells |
| Q45300176 | Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfunction in Huntington's Disease. |
| Q36482299 | Histone deacetylase (HDAC) inhibitors targeting HDAC3 and HDAC1 ameliorate polyglutamine-elicited phenotypes in model systems of Huntington's disease. |
| Q40152899 | Histone deacetylase 6 inhibition compensates for the transport deficit in Huntington's disease by increasing tubulin acetylation |
| Q45298333 | History of genetic disease: the molecular genetics of Huntington disease - a history |
| Q35867566 | Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD. |
| Q64246810 | Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes |
| Q33594204 | Huntingtin facilitates polycomb repressive complex 2. |
| Q24337727 | Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity |
| Q45294110 | Huntingtin interacting protein HYPK is a negative regulator of heat shock response and is downregulated in models of Huntington's Disease |
| Q28189644 | Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes |
| Q24294765 | Huntingtin is required for mitotic spindle orientation and mammalian neurogenesis |
| Q36981055 | Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner |
| Q39106346 | Huntingtin polyQ Mutation Impairs the 17β-Estradiol/Neuroglobin Pathway Devoted to Neuron Survival. |
| Q38447132 | Huntingtin promotes mTORC1 signaling in the pathogenesis of Huntington's disease. |
| Q34274478 | Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis. |
| Q36098669 | Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation. |
| Q36839701 | Huntingtin's spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function |
| Q30480364 | Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease |
| Q56602105 | Huntingtin: Alive and Well and Working in Middle Management |
| Q28182055 | Huntingtin: alive and well and working in middle management |
| Q37053914 | Huntington's Disease Protein Huntingtin Associates with its own mRNA. |
| Q28365461 | Huntington's disease |
| Q79121423 | Huntington's disease |
| Q37996350 | Huntington's disease and the striatal medium spiny neuron: cell-autonomous and non-cell-autonomous mechanisms of disease |
| Q36825291 | Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies. |
| Q37889820 | Huntington's disease, calcium, and mitochondria |
| Q35946323 | Huntington's disease: Neural dysfunction linked to inositol polyphosphate multikinase |
| Q33752885 | Identification and evaluation of small molecule pan-caspase inhibitors in Huntington's disease models |
| Q33883037 | Identification and validation of novel spinophilin-associated proteins in rodent striatum using an enhanced ex vivo shotgun proteomics approach |
| Q34525270 | Identification of HYPK-interacting proteins reveals involvement of HYPK in regulating cell growth, cell cycle, unfolded protein response and cell death |
| Q41701052 | Identifying therapeutic targets by combining transcriptional data with ordinal clinical measurements. |
| Q42042260 | Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity |
| Q36159463 | Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease |
| Q33967163 | Impaired TrkB-mediated ERK1/2 activation in huntington disease knock-in striatal cells involves reduced p52/p46 Shc expression |
| Q35311070 | Impaired alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor trafficking and function by mutant huntingtin. |
| Q39726453 | Impaired ganglioside metabolism in Huntington's disease and neuroprotective role of GM1. |
| Q34278252 | Impaired heat shock response in cells expressing full-length polyglutamine-expanded huntingtin |
| Q27312215 | Impaired mitochondrial dynamics and Nrf2 signaling contribute to compromised responses to oxidative stress in striatal cells expressing full-length mutant huntingtin |
| Q47844404 | In silico probing and biological evaluation of SETDB1/ESET-targeted novel compounds that reduce tri-methylated histone H3K9 (H3K9me3) level |
| Q35552239 | Increased 90-kDa ribosomal S6 kinase (Rsk) activity is protective against mutant huntingtin toxicity. |
| Q49823914 | Increased Levels of Rictor Prevent Mutant Huntingtin-Induced Neuronal Degeneration. |
| Q39952286 | Increased expression of Bim contributes to the potentiation of serum deprivation-induced apoptotic cell death in Huntington's disease knock-in striatal cell line |
| Q37857439 | Induced pluripotent stem cells: a new revolution for clinical neurology? |
| Q39885837 | Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction |
| Q37592772 | Inefficient degradation of truncated polyglutamine proteins by the proteasome |
| Q36100251 | Inhibition of Excessive Monoamine Oxidase A/B Activity Protects Against Stress-induced Neuronal Death in Huntington Disease. |
| Q46236725 | Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein. |
| Q45299979 | Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421. |
| Q34236510 | Inhibition of lipid signaling enzyme diacylglycerol kinase epsilon attenuates mutant huntingtin toxicity |
| Q37384081 | Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration |
| Q28394803 | Inhibition of mitochondrial protein import by mutant huntingtin |
| Q34743269 | Inhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease. |
| Q28507004 | Integration of β-catenin, sirtuin, and FOXO signaling protects from mutant huntingtin toxicity |
| Q36319566 | Interrogation of brain miRNA and mRNA expression profiles reveals a molecular regulatory network that is perturbed by mutant huntingtin |
| Q35023030 | Iron accumulates in Huntington's disease neurons: protection by deferoxamine |
| Q26829932 | Iron dysregulation in Huntington's disease |
| Q99629125 | Isomeric O-methyl cannabidiolquinones with dual BACH1/NRF2 activity |
| Q39533990 | Kinase inhibitors modulate huntingtin cell localization and toxicity. |
| Q36533102 | Knockdown of the small conductance Ca(2+) -activated K(+) channels is potently cytotoxic in breast cancer cell lines |
| Q90684471 | Lipid Membranes Influence the Ability of Small Molecules To Inhibit Huntingtin Fibrillization |
| Q39014909 | Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain. |
| Q91715150 | Loss of Mevalonate/Cholesterol Homeostasis in the Brain: A Focus on Autism Spectrum Disorder and Rett Syndrome |
| Q45305379 | Loss of huntingtin function complemented by small molecules acting as repressor element 1/neuron restrictive silencer element silencer modulators |
| Q42478056 | Loss of striatal type 1 cannabinoid receptors is a key pathogenic factor in Huntington's disease. |
| Q38580123 | Main path and byways: non-vesicular glutamate release by system xc(-) as an important modifier of glutamatergic neurotransmission |
| Q36622998 | Manganese transport via the transferrin mechanism |
| Q30842363 | Mapping Cannabinoid 1 Receptor Allosteric Site(s): Critical Molecular Determinant and Signaling Profile of GAT100, a Novel, Potent, and Irreversibly Binding Probe |
| Q39673821 | Matrix Metalloproteinases Are Modifiers of Huntingtin Proteolysis and Toxicity in Huntington's Disease |
| Q37508593 | MeCP2: a novel Huntingtin interactor |
| Q38009700 | Mechanism(s) of alteration of micro RNA expressions in Huntington's disease and their possible contributions to the observed cellular and molecular dysfunctions in the disease |
| Q34424265 | Meclizine is neuroprotective in models of Huntington's disease |
| Q90390323 | Melatonin inhibits cytosolic mitochondrial-DNA induced neuroinflammatory signaling in accelerated aging and neurodegeneration |
| Q24791822 | Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis |
| Q34140579 | Metabolic state determines sensitivity to cellular stress in Huntington disease: normalization by activation of PPARγ. |
| Q33817346 | Metformin intake associates with better cognitive function in patients with Huntington's disease. |
| Q35034398 | Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches |
| Q37178581 | Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease |
| Q37355444 | Mitochondrial DNA damage is associated with reduced mitochondrial bioenergetics in Huntington's disease. |
| Q38866668 | Mitochondrial death functions of p53. |
| Q41998875 | Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease |
| Q21560776 | Mitochondrial dysfunction in Pten haplo-insufficient mice with social deficits and repetitive behavior: interplay between Pten and p53 |
| Q34605764 | Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli |
| Q37427280 | Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease. |
| Q45297269 | Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin |
| Q45302980 | Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors. |
| Q33575494 | Modeling Huntington disease in yeast: perspectives and future directions |
| Q34408222 | Modeling Huntington's disease in cells, flies, and mice |
| Q39567922 | Modeling pathogenesis of Huntington's disease with inducible neuroprogenitor cells |
| Q96228741 | Modulation of dopamine D1 receptors via histamine H3 receptors is a novel therapeutic target for Huntington's disease |
| Q39626047 | Modulation of mutant huntingtin N-terminal cleavage and its effect on aggregation and cell death. |
| Q44573861 | Modulation of nuclear REST by alternative splicing: a potential therapeutic target for Huntington's disease |
| Q37638953 | Molecular identification of ancient and modern mammalian magnesium transporters |
| Q28576523 | Mutant Huntingtin Alters Cell Fate in Response to Microtubule Depolymerization via the GEF-H1-RhoA-ERK Pathway |
| Q39168396 | Mutant Huntingtin alters retrograde transport of TrkB receptors in striatal dendrites. |
| Q38458943 | Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling |
| Q45300380 | Mutant huntingtin affects cortical progenitor cell division and development of the mouse neocortex |
| Q34829422 | Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease |
| Q92185503 | Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23 |
| Q40231756 | Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences |
| Q30486228 | Mutant huntingtin impairs post-Golgi trafficking to lysosomes by delocalizing optineurin/Rab8 complex from the Golgi apparatus |
| Q40199654 | Mutant huntingtin impairs the post-Golgi trafficking of brain-derived neurotrophic factor but not its Val66Met polymorphism. |
| Q55711820 | Mutant huntingtin induces iron overload via up-regulating IRP1 in Huntington's disease. |
| Q38618002 | Mutant huntingtin is secreted via a late endosomal/lysosomal unconventional secretory pathway |
| Q94948425 | Mutations in the KIF21B kinesin gene cause neurodevelopmental disorders through imbalanced canonical motor activity |
| Q89480421 | N6-Furfuryladenine is protective in Huntington's disease models by signaling huntingtin phosphorylation |
| Q37684006 | Nanomedicine-based neuroprotective strategies in patient specific-iPSC and personalized medicine |
| Q99633330 | Neural stem cells derived from the developing forebrain of YAC128 mice exhibit pathological features of Huntington's disease |
| Q42109654 | Neurodegeneration in Huntington's disease involves loss of cystathionine γ-lyase |
| Q89531500 | Neuronal L-Type Calcium Channel Signaling to the Nucleus Requires a Novel CaMKIIα-Shank3 Interaction |
| Q44607270 | Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport |
| Q39519656 | Neuroprotective effects of white tea against oxidative stress-induced toxicity in striatal cells. |
| Q34440282 | Neuroprotective properties of cannabigerol in Huntington's disease: studies in R6/2 mice and 3-nitropropionate-lesioned mice. |
| Q42664318 | New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins |
| Q28281904 | Normal huntingtin function: an alternative approach to Huntington's disease |
| Q39607494 | Novel high-throughput assay to assess cellular manganese levels in a striatal cell line model of Huntington's disease confirms a deficit in manganese accumulation |
| Q35108405 | Novel inhibitors of mitochondrial sn-glycerol 3-phosphate dehydrogenase. |
| Q37174262 | Optimization of fluorescence assay of cellular manganese status for high throughput screening |
| Q45294879 | Oxidizing effects of exogenous stressors in Huntington's disease knock-in striatal cells--protective effect of cystamine and creatine |
| Q38100286 | PABPN1: molecular function and muscle disease |
| Q30583566 | PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function. |
| Q38680458 | PIN1 Modulates Huntingtin Levels and Aggregate Accumulation: An In vitro Model |
| Q36347184 | PINK1-induced mitophagy promotes neuroprotection in Huntington's disease |
| Q47322495 | PPARδ activation by bexarotene promotes neuroprotection by restoring bioenergetic and quality control homeostasis |
| Q43207028 | PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD). |
| Q36283269 | Palmitoylation and trafficking of GAD65 are impaired in a cellular model of Huntington's disease |
| Q45299287 | Palmitoylation of caspase-6 by HIP14 regulates its activation |
| Q38698653 | Pharmacological evidence for a metabotropic glutamate receptor heterodimer in neuronal cells |
| Q64084736 | Phase-to-Phase With Nucleoli - Stress Responses, Protein Aggregation and Novel Roles of RNA |
| Q40111505 | Phosphorylation of huntingtin by cyclin-dependent kinase 5 is induced by DNA damage and regulates wild-type and mutant huntingtin toxicity in neurons. |
| Q39943159 | Phosphorylation of mutant huntingtin at S421 restores anterograde and retrograde transport in neurons. |
| Q28484973 | Pitfalls in the detection of cholesterol in Huntington's disease models |
| Q36588268 | Pizotifen Activates ERK and Provides Neuroprotection in vitro and in vivo in Models of Huntington's Disease |
| Q36509945 | Poly-glutamine expanded huntingtin dramatically alters the genome wide binding of HSF1. |
| Q40985899 | Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease models |
| Q78315034 | Polyglutamine protein aggregates are dynamic |
| Q38818577 | Polyglutamine tracts regulate beclin 1-dependent autophagy |
| Q38374920 | Post-Translational Modifications (PTMs), Identified on Endogenous Huntingtin, Cluster within Proteolytic Domains between HEAT Repeats |
| Q38788190 | Potential Transfer of Polyglutamine and CAG-Repeat RNA in Extracellular Vesicles in Huntington's Disease: Background and Evaluation in Cell Culture |
| Q30499096 | Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment. |
| Q34392509 | Protection by glia-conditioned medium in a cell model of Huntington disease |
| Q30418699 | Proteomic analysis of wild-type and mutant huntingtin-associated proteins in mouse brains identifies unique interactions and involvement in protein synthesis |
| Q48200890 | RTP801 Is Involved in Mutant Huntingtin-Induced Cell Death |
| Q42382351 | Real-time imaging of Huntingtin aggregates diverting target search and gene transcription |
| Q30446460 | Redox proteomics in selected neurodegenerative disorders: from its infancy to future applications. |
| Q35874721 | Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease |
| Q45305834 | Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease. |
| Q39240888 | Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models. |
| Q38674322 | Regulation of RE1 protein silencing transcription factor (REST) expression by HIP1 protein interactor (HIPPI). |
| Q34009621 | Regulation of miR-146a by RelA/NFkB and p53 in STHdh(Q111)/Hdh(Q111) cells, a cell model of Huntington's disease |
| Q27306293 | Reinstating aberrant mTORC1 activity in Huntington's disease mice improves disease phenotypes |
| Q48771662 | Remodeling of heterochromatin structure slows neuropathological progression and prolongs survival in an animal model of Huntington's disease. |
| Q38339107 | Rescue of gene expression by modified REST decoy oligonucleotides in a cellular model of Huntington's disease |
| Q34406809 | Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons. |
| Q39538215 | Revealing disease-associated pathways by network integration of untargeted metabolomics. |
| Q24799882 | Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation |
| Q40674805 | Reversal of senescence in mouse fibroblasts through lentiviral suppression of p53. |
| Q91929130 | Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion |
| Q24317318 | Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity |
| Q37941268 | Role of manganese in neurodegenerative diseases |
| Q37557789 | Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease |
| Q28386202 | Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis |
| Q42434605 | Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease |
| Q24310080 | Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease |
| Q34579818 | Single nucleotide seed modification restores in vivo tolerability of a toxic artificial miRNA sequence in the mouse brain |
| Q39013000 | Singular Location and Signaling Profile of Adenosine A2A-Cannabinoid CB1 Receptor Heteromers in the Dorsal Striatum. |
| Q52692554 | Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease. |
| Q39064401 | Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress |
| Q33578074 | Striatal neurodevelopment is dysregulated in purine metabolism deficiency and impacts DARPP-32, BDNF/TrkB expression and signaling: new insights on the molecular and cellular basis of Lesch-Nyhan Syndrome |
| Q34994813 | Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis |
| Q38165760 | Studying neurodegenerative diseases in culture models. |
| Q45305579 | Suppression of MAPK11 or HIPK3 reduces mutant Huntingtin levels in Huntington's disease models |
| Q47393947 | Sustained Gq-Protein Signaling Disrupts Striatal Circuits via JNK. |
| Q36389503 | Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice |
| Q24292042 | TBX-3, the gene mutated in Ulnar-Mammary Syndrome, is a negative regulator of p19ARF and inhibits senescence |
| Q38925490 | Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease |
| Q52613225 | Targeting Gpr52 lowers mutant HTT levels and rescues Huntington's disease-associated phenotypes. |
| Q64057387 | Targeting the neuronal calcium sensor DREAM with small-molecules for Huntington's disease treatment |
| Q38957994 | Tau hyperphosphorylation and deregulation of calcineurin in mouse models of Huntington's disease. |
| Q40075003 | Tetrahydrocannabinolic acid is a potent PPARγ agonist with neuroprotective activity. |
| Q29306901 | The CB1 cannabinoid receptor signals striatal neuroprotection via a PI3K/Akt/mTORC1/BDNF pathway |
| Q99410963 | The Neurodevelopmental Hypothesis of Huntington's Disease |
| Q39213276 | The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity. |
| Q27314681 | The Wnt receptor Ryk reduces neuronal and cell survival capacity by repressing FOXO activity during the early phases of mutant huntingtin pathogenicity |
| Q45290121 | The biology of Huntington's disease. |
| Q40402039 | The calpain-suppressing effects of olesoxime in Huntington's disease. |
| Q41967848 | The challenge in translating basic research discoveries to treatment of Huntington disease |
| Q34496600 | The common inhaled anesthetic isoflurane increases aggregation of huntingtin and alters calcium homeostasis in a cell model of Huntington's disease |
| Q37441183 | The failure of mitochondria leads to neurodegeneration: Do mitochondria need a jump start? |
| Q44376761 | The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. |
| Q34042999 | The histone demethylase Jarid1b (Kdm5b) is a novel component of the Rb pathway and associates with E2f-target genes in MEFs during senescence |
| Q41154714 | The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal |
| Q36873629 | The interaction of polyglutamine peptides with lipid membranes is regulated by flanking sequences associated with huntingtin |
| Q37765841 | The interrelationship between mitochondrial dysfunction and transcriptional dysregulation in Huntington disease |
| Q97590555 | The longevity-associated variant of BPIFB4 improves a CXCR4-mediated striatum-microglia crosstalk preventing disease progression in a mouse model of Huntington's disease |
| Q28591685 | The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation |
| Q39235671 | The role of the cofilin-actin rod stress response in neurodegenerative diseases uncovers potential new drug targets |
| Q33822501 | The stress response factor daf-16/FOXO is required for multiple compound families to prolong the function of neurons with Huntington's disease |
| Q47442924 | Towards an Understanding of Energy Impairment in Huntington's Disease Brain |
| Q36957020 | Toxicity of the flame-retardant BDE-49 on brain mitochondria and neuronal progenitor striatal cells enhanced by a PTEN-deficient background |
| Q37161760 | Transcriptional control of amino acid homeostasis is disrupted in Huntington's disease |
| Q36190256 | Transcriptional regulation of microRNA-100, -146a, and -150 genes by p53 and NFκB p65/RelA in mouse striatal STHdh(Q7)/ Hdh(Q7) cells and human cervical carcinoma HeLa cells |
| Q36379946 | Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease |
| Q35268092 | Truncated peroxisome proliferator-activated receptor-γ coactivator 1α splice variant is severely altered in Huntington's disease |
| Q34138944 | Type 1 cannabinoid receptor ligands display functional selectivity in a cell culture model of striatal medium spiny projection neurons |
| Q42510495 | Type 2 transglutaminase differentially modulates striatal cell death in the presence of wild type or mutant huntingtin |
| Q36239473 | Ubiquitin Accumulation on Disease Associated Protein Aggregates Is Correlated with Nuclear Ubiquitin Depletion, Histone De-Ubiquitination and Impaired DNA Damage Response |
| Q28469280 | Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism |
| Q35079642 | Untargeted metabolic profiling identifies interactions between Huntington's disease and neuronal manganese status |
| Q28483257 | Using FLIM-FRET to measure conformational changes of transglutaminase type 2 in live cells |
| Q45299293 | VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease |
| Q37223339 | VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease |
| Q39333674 | Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease. |
| Q39279550 | hMTH1 expression protects mitochondria from Huntington's disease-like impairment |
| Q24316303 | pARIS-htt: an optimised expression platform to study huntingtin reveals functional domains required for vesicular trafficking |
| Q36098005 | trans-(-)-ε-Viniferin increases mitochondrial sirtuin 3 (SIRT3), activates AMP-activated protein kinase (AMPK), and protects cells in models of Huntington Disease |
| Q54970579 | STHdhQ111 | described by source | P1343 |
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