Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

scientific article published in November 2007

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1093/HMG/DDM217
P3181OpenCitations bibliographic resource ID4626525
P698PubMed publication ID17704510
P5875ResearchGate publication ID6134339

P50authorRichard M. EpandQ43656907
P2093author name stringJianrun Xia
Jillian Taylor
Ray Truant
Deborah Pinchev
Randy Singh Atwal
P2860cites workHuntingtin contains a highly conserved nuclear export signalQ24303895
Huntingtin interacting protein 1 modulates the transcriptional activity of nuclear hormone receptorsQ24308715
Differential interaction of nuclear receptors with the putative human transcriptional coactivator hTIF1Q24315063
Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neuronsQ24317574
SUMO modification of Huntingtin and Huntington's disease pathologyQ24324137
Visualization of Rab9-mediated vesicle transport from endosomes to the trans-Golgi in living cellsQ24673272
Induction of autophagy causes dramatic changes in the subcellular distribution of GFP-Rab24.Q44016477
Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in DrosophilaQ44607268
Monoclonal antibodies from three new regions of huntingtin, the Huntington's disease proteinQ45293875
Neuropathological classification of Huntington's diseaseQ45297167
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtinQ45297223
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.Q45298582
Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.Q45301281
Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cellsQ45301722
HEAT repeats in the Huntington's disease proteinQ45307276
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.Q48373564
Detection of autophagy in tissue by standard immunohistochemistry: possibilities and limitations.Q50721155
Mouse Models of Triplet Repeat DiseasesQ57877560
Gain of glutamines, gain of function?Q71990830
Live-cell nucleocytoplasmic protein shuttle assay utilizing laser confocal microscopy and FRAPQ77540431
Differential localization of Rho GTPases in live cells: regulation by hypervariable regions and RhoGDI bindingQ24674800
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genesQ28189644
Loss of huntingtin-mediated BDNF gene transcription in Huntington's diseaseQ28202050
Nrf1 is targeted to the endoplasmic reticulum membrane by an N-terminal transmembrane domain. Inhibition of nuclear translocation and transacting functionQ28239381
LC3 conjugation system in mammalian autophagyQ28278334
Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal deathQ28287762
Comparative genomics, evolution and origins of the nuclear envelope and nuclear pore complexQ28299432
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in miceQ28505664
Deletion of the triplet repeat encoding polyglutamine within the mouse Huntington's disease gene results in subtle behavioral/motor phenotypes in vivo and elevated levels of ATP with cellular senescence in vitroQ28510315
siRNA-mediated inhibition of endogenous Huntington disease gene expression induces an aberrant configuration of the ER network in vitroQ28510833
Dynamics of intracellular granules with CD63-GFP in rat basophilic leukemia cellsQ28576303
Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organellesQ28589989
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubulesQ28590634
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic miceQ29615357
Comparison of ARM and HEAT protein repeatsQ29616046
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brainQ29617982
Rab7: a key to lysosome biogenesisQ29620369
An amino-terminal amphipathic alpha-helix mediates membrane association of the hepatitis C virus nonstructural protein 5AQ29620628
Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.Q30164317
Computer-assisted image analysis protocol that quantitatively measures subnuclear protein organization in cell populationsQ30312129
Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's diseaseQ30480364
Interaction of huntingtin fragments with brain membranes--clues to early dysfunction in Huntington's diseaseQ33231356
Recent insights into the molecular pathogenesis of Huntington diseaseQ33862546
Identification of the endoplasmic reticulum targeting signal in vesicle-associated membrane proteins.Q33883841
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivoQ34044164
Intracellular signaling from the endoplasmic reticulum to the nucleus: the unfolded protein response in yeast and mammalsQ34245400
Flanking sequences profoundly alter polyglutamine toxicity in yeastQ34984292
Wild-type huntingtin plays a role in brain development and neuronal survivalQ35622306
Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesisQ35751839
Beyond lectins: the calnexin/calreticulin chaperone system of the endoplasmic reticulumQ36391681
Endoplasmic reticulum stress in health and diseaseQ36510035
Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionationQ36823776
Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease.Q36854847
Autophagosome-like vacuole formation in Huntington's disease lymphoblastsQ42462336
Ataxin-7 can export from the nucleus via a conserved exportin-dependent signal.Q42807632
P433issue21
P407language of work or nameEnglishQ1860
P921main subjectHuntingtinQ2620886
P304page(s)2600-2615
P577publication date2007-08-18
P1433published inHuman Molecular GeneticsQ2720965
P1476titleHuntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity
P478volume16

Reverse relations

cites work (P2860)
Q42395501A Plasma Membrane Association Module in Yeast Amino Acid Transporters
Q47787775A comprehensive in silico analysis of huntingtin and its interactome
Q37157263A flexible polyglutamine hinge opens new doors for understanding huntingtin function
Q27301289A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease.
Q39358037A multifunctional, multi-pathway intracellular localization signal in Huntingtin.
Q64882921A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths.
Q45290293A study of molecular changes relating to energy metabolism and cellular stress in people with Huntington's disease: looking for biomarkers
Q34627072Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage
Q48822102Accumulation of the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases
Q37142133Acetylation within the First 17 Residues of Huntingtin Exon 1 Alters Aggregation and Lipid Binding.
Q41851500Altered Co-Translational Processing Plays a Role in Huntington's Pathogenesis-A Hypothesis
Q64931945Altered lactate metabolism in Huntington's disease is dependent on GLUT3 expression.
Q35912172Altered lysosomal positioning affects lysosomal functions in a cellular model of Huntington's disease
Q30385936An Intein-based Strategy for the Production of Tag-free Huntingtin Exon 1 Proteins Enables New Insights into the Polyglutamine Dependence of Httex1 Aggregation and Fibril Formation
Q36647206An N-terminal nuclear export signal regulates trafficking and aggregation of Huntingtin (Htt) protein exon 1.
Q37810562Antibody Therapy in Neurodegenerative Disease
Q44962020Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy
Q45297035Atomistic mechanisms of huntingtin N-terminal fragment insertion on a phospholipid bilayer revealed by molecular dynamics simulations
Q28084804Autophagosome dynamics in neurodegeneration at a glance
Q38528206Autophagy and Lipid Droplets in the Liver
Q37935233Autophagy and polyglutamine diseases
Q27011387Autophagy in polyglutamine disease: Imposing order on disorder or contributing to the chaos?
Q27676093Beta conformation of polyglutamine track revealed by a crystal structure of Huntingtin N-terminal region with insertion of three histidine residues
Q34117333Bifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments
Q30427825Biophysical insights into how surfaces, including lipid membranes, modulate protein aggregation related to neurodegeneration.
Q35703870CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse
Q42353203CCT complex restricts neuropathogenic protein aggregation via autophagy
Q33814719Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease
Q57246845Chaperone-Mediated Autophagy and Parkinson's Disease
Q30787791Cholesterol Modifies Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes
Q36588291Conformation sensors that distinguish monomeric proteins from oligomers in live cells
Q92777658Conformational studies of pathogenic expanded polyglutamine protein deposits from Huntington's disease
Q33459255Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity
Q61135436Contact inhibition controls cell survival and proliferation via YAP/TAZ-autophagy axis
Q58994518Crosstalk between Endoplasmic Reticulum Stress and Protein Misfolding in Neurodegenerative Diseases
Q37760791Current understanding on the pathogenesis of polyglutamine diseases
Q34752467Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin
Q30437121Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice.
Q30425063Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice
Q37571975Developing intrabodies for the therapeutic suppression of neurodegenerative pathology
Q35052612Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells
Q34313192Differential nuclear localization of complexes may underlie in vivo intrabody efficacy in Huntington's disease
Q35641611Discovery of novel isoforms of huntingtin reveals a new hominid-specific exon
Q34618914Does Huntingtin play a role in selective macroautophagy?
Q41822886Early and late events induced by polyQ-expanded proteins: identification of a common pathogenic property of polYQ-expanded proteins
Q34112214Early autophagic response in a novel knock-in model of Huntington disease
Q37961564Engineered antibody therapies to counteract mutant huntingtin and related toxic intracellular proteins
Q37962584Epigenetic treatment of neurological disease
Q44933645Essential sequence of the N-terminal cytoplasmic localization-related domain of huntingtin and its effect on huntingtin aggregates
Q47558279Evaluation of Cognition and Cortical Excitability in Huntington's Disease
Q33530121F-actin binding regions on the androgen receptor and huntingtin increase aggregation and alter aggregate characteristics
Q33761247Fibril polymorphism affects immobilized non-amyloid flanking domains of huntingtin exon1 rather than its polyglutamine core
Q31055925Free-Energy Landscape of the Amino-Terminal Fragment of Huntingtin in Aqueous Solution
Q38259591From pathways to targets: understanding the mechanisms behind polyglutamine disease.
Q39877377GFP-LC3 labels organised smooth endoplasmic reticulum membranes independently of autophagy
Q30506469Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice
Q30353131Generation and Characterization of Knock-in Mouse Models Expressing Versions of Huntingtin with Either an N17 or a Combined PolyQ and Proline-Rich Region Deletion.
Q64896030Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy.
Q37165188Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis
Q27023050Genetics and neuropathology of Huntington's disease
Q35014895Grb2 is regulated by foxd3 and has roles in preventing accumulation and aggregation of mutant huntingtin
Q90411062High-mobility group box 1 links sensing of reactive oxygen species by huntingtin to its nuclear entry
Q41731654Huntingtin N17 domain is a reactive oxygen species sensor regulating huntingtin phosphorylation and localization
Q35867566Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD.
Q41881745Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors
Q30498164Huntingtin coordinates the dynein-mediated dynamic positioning of endosomes and lysosomes
Q33529116Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP.
Q45299796Huntingtin is a scaffolding protein in the ATM oxidative DNA damage response complex.
Q36098669Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation.
Q56083219Huntington disease
Q42472433Huntington's disease and mitochondrial alterations: emphasis on experimental models
Q45303341Huntington's disease: flipping a switch on huntingtin
Q34797845Huntington's disease: revisiting the aggregation hypothesis in polyglutamine neurodegenerative diseases
Q30435758IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.
Q30447129Identification of a karyopherin β1/β2 proline-tyrosine nuclear localization signal in huntingtin protein
Q30578636Identification of a post-translationally myristoylated autophagy-inducing domain released by caspase cleavage of huntingtin.
Q89135698Identification of distinct conformations associated with monomers and fibril assemblies of mutant huntingtin
Q99614732Implications of the Orb2 Amyloid Structure in Huntington's Disease
Q34313991Increased mitochondrial fission and neuronal dysfunction in Huntington's disease: implications for molecular inhibitors of excessive mitochondrial fission
Q39426587Inhibiting the nucleation of amyloid structure in a huntingtin fragment by targeting α-helix-rich oligomeric intermediates
Q39943981Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity
Q44742505Investigation of membrane penetration depth and interactions of the amino-terminal domain of huntingtin: refined analysis by tryptophan fluorescence measurement
Q26829932Iron dysregulation in Huntington's disease
Q30353134Is Huntingtin Dispensable in the Adult Brain?
Q39533990Kinase inhibitors modulate huntingtin cell localization and toxicity.
Q36088674Kinetically competing huntingtin aggregation pathways control amyloid polymorphism and properties
Q35940409Latrepirdine, a potential novel treatment for Alzheimer's disease and Huntington's chorea
Q90684471Lipid Membranes Influence the Ability of Small Molecules To Inhibit Huntingtin Fibrillization
Q45008320Lysine residues in the N-terminal huntingtin amphipathic α-helix play a key role in peptide aggregation
Q45290192Mass Spectrometry Analysis of Wild-Type and Knock-in Q140/Q140 Huntington's Disease Mouse Brains Reveals Changes in Glycerophospholipids Including Alterations in Phosphatidic Acid and Lyso-Phosphatidic Acid
Q30378040Membrane Curvature-sensing and Curvature-inducing Activity of Islet Amyloid Polypeptide and Its Implications for Membrane Disruption.
Q38974698Mitochondria-Associated Membranes (MAMs): Overview and Its Role in Parkinson's Disease
Q41998875Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease
Q37356257Mitochondrial structural and functional dynamics in Huntington's disease
Q37776087Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Q35048841Molecular interaction between the chaperone Hsc70 and the N-terminal flank of huntingtin exon 1 modulates aggregation
Q33788913Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion
Q42089641Mutant A53T alpha-synuclein induces neuronal death by increasing mitochondrial autophagy
Q37308357Mutant huntingtin and mitochondrial dysfunction
Q34829422Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease
Q92185503Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23
Q55711820Mutant huntingtin induces iron overload via up-regulating IRP1 in Huntington's disease.
Q35747131Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease
Q35645271Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease
Q64944850N-terminal Huntingtin (Htt) phosphorylation is a molecular switch regulating Htt aggregation, helical conformation, internalization, and nuclear targeting.
Q27306025N17 Modifies mutant Huntingtin nuclear pathogenesis and severity of disease in HD BAC transgenic mice
Q89480421N6-Furfuryladenine is protective in Huntington's disease models by signaling huntingtin phosphorylation
Q35572092Neurodegenerative processes in Huntington's disease
Q38074521Neuronal Ca(2+) dyshomeostasis in Huntington disease.
Q45341843One-pot semisynthesis of exon 1 of the Huntingtin protein: new tools for elucidating the role of posttranslational modifications in the pathogenesis of Huntington's disease
Q33815642Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1
Q45305618Phosphorylation of huntingtin at residue T3 is decreased in Huntington's disease and modulates mutant huntingtin protein conformation
Q37447680Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity
Q37981477Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence
Q34414446Polyglutamine amyloid core boundaries and flanking domain dynamics in huntingtin fragment fibrils determined by solid-state nuclear magnetic resonance.
Q42904942Polyglutamine dances the conformational cha-cha-cha
Q43100835Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism
Q37157299Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin
Q33818809Polyglutamine induced misfolding of huntingtin exon1 is modulated by the flanking sequences
Q35479250Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy
Q38374920Post-Translational Modifications (PTMs), Identified on Endogenous Huntingtin, Cluster within Proteolytic Domains between HEAT Repeats
Q57789346PreSMo Target-Binding Signatures in Intrinsically Disordered Proteins
Q35096897Prevalent polymorphism in thyroid hormone-activating enzyme leaves a genetic fingerprint that underlies associated clinical syndromes
Q51238345Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease.
Q35221894Probing the Huntingtin 1-17 membrane anchor on a phospholipid bilayer by using all-atom simulations
Q47767382Protein phosphatase 1 regulates huntingtin exon 1 aggregation and toxicity.
Q39124557Proteins Containing Expanded Polyglutamine Tracts and Neurodegenerative Disease
Q30418699Proteomic analysis of wild-type and mutant huntingtin-associated proteins in mouse brains identifies unique interactions and involvement in protein synthesis
Q28506165Proteotoxic stress increases nuclear localization of ataxin-3
Q40355188Prothymosin-α interacts with mutant huntingtin and suppresses its cytotoxicity in cell culture.
Q35005658RNAi screening in Drosophila cells identifies new modifiers of mutant huntingtin aggregation
Q88316783Redox Mechanisms in Neurodegeneration: From Disease Outcomes to Therapeutic Opportunities
Q37696972Repeat expansion disease: progress and puzzles in disease pathogenesis
Q38743344Role of Sigma-1 Receptor in Cocaine Abuse and Neurodegenerative Disease.
Q28544898Scalable production in human cells and biochemical characterization of full-length normal and mutant huntingtin
Q27657408Secondary Structure of Huntingtin Amino-Terminal Region
Q41520412Serine phosphorylation suppresses huntingtin amyloid accumulation by altering protein aggregation properties.
Q33594383Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice
Q36600026Slow amyloid nucleation via α-helix-rich oligomeric intermediates in short polyglutamine-containing huntingtin fragments.
Q52692554Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease.
Q49331880Sphingomyelin and GM1 Influence Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes
Q27679485Structure and Topology of the Huntingtin 1–17 Membrane Anchor by a Combined Solution and Solid-State NMR Approach
Q41518158Structure of a single-chain Fv bound to the 17 N-terminal residues of huntingtin provides insights into pathogenic amyloid formation and suppression.
Q30610715Super-resolution fluorescence of huntingtin reveals growth of globular species into short fibers and coexistence of distinct aggregates
Q88299111Tadpole-like Conformations of Huntingtin Exon 1 Are Characterized by Conformational Heterogeneity that Persists regardless of Polyglutamine Length
Q38019159Targeting Huntington's disease through histone deacetylases
Q91899926Targeting N-Terminal Huntingtin with a Dual-sgRNA Strategy by CRISPR/Cas9
Q37774037Targeting histone deacetylases for the treatment of Huntington's disease.
Q35909430Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy
Q47251076The 17-residue-long N terminus in huntingtin controls step-wise aggregation in solution and on membranes via different mechanisms
Q36694395The 2.2-Angstrom resolution crystal structure of the carboxy-terminal region of ataxin-3.
Q27301298The N17 domain mitigates nuclear toxicity in a novel zebrafish Huntington's disease model
Q33838789The aggregation-enhancing huntingtin N-terminus is helical in amyloid fibrils
Q37969749The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology
Q42699018The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation
Q50551501The composition of the polyglutamine-containing proteins influences their co-aggregation properties
Q36110838The emerging role of the first 17 amino acids of huntingtin in Huntington's disease
Q41154714The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal
Q36873629The interaction of polyglutamine peptides with lipid membranes is regulated by flanking sequences associated with huntingtin
Q26823365The many faces of autophagy dysfunction in Huntington's disease: from mechanism to therapy
Q28591685The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation
Q39235671The role of the cofilin-actin rod stress response in neurodegenerative diseases uncovers potential new drug targets
Q38544332The unfolded protein response and its potential role in Huntington's disease elucidated by a systems biology approach.
Q37825926The unfolded protein response: how protein folding became a restrictive aspect for innate immunity and B lymphocytes
Q64859904Therapeutic approaches to Huntington disease: from the bench to the clinic
Q44087369Toxicity and endocytosis of spinocerebellar ataxia type 6 polyglutamine domains: role of myosin IIb.
Q28742836Unusual structures are present in DNA fragments containing super-long Huntingtin CAG repeats
Q52671146Wolfram syndrome: MAMs' connection?

Search more.