Mutant Huntingtin Protein Interaction Map Implicates Dysregulation of Multiple Cellular Pathways in Neurodegeneration of Huntington’s Disease

Mutant Huntingtin stalls ribosomes and represses protein synthesis in a cellular model of Huntington disease

(2021) Mehdi Eshraghi et al.   Nature Communications

HDinHD: A Rich Data Portal for Huntington’s Disease Research

(2021) Jeff Aaronson et al.   Journal of Huntingtons Disease

Identification of Full-Length Wild-Type and Mutant Huntingtin Interacting Proteins by Crosslinking Immunoprecipitation in Mice Brain Cortex

(2021) Karen A. Sap et al.   Journal of Huntingtons Disease

Huntingtin Ubiquitination Mechanisms and Novel Possible Therapies to Decrease the Toxic Effects of Mutated Huntingtin

(2021) Annarita Fiorillo et al.   Journal of Personalized Medicine

Differential effects of SNARE-dependent gliotransmission on behavioral phenotypes in a mouse model of Huntington's disease

(2020) Annesha C. King et al.   EXPERIMENTAL NEUROLOGY

The Gene Ontology resource: enriching a GOld mine

(2020) et al.   NUCLEIC ACIDS RESEARCH

The STRING database in 2021: customizable protein–protein networks, and functional characterization of user-uploaded gene/measurement sets

(2020) Damian Szklarczyk et al.   NUCLEIC ACIDS RESEARCH

Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease

(2019) Judith Schilling et al.   JOURNAL OF MOLECULAR BIOLOGY

PolyQ-expanded huntingtin and ataxin-3 sequester ubiquitin adaptors hHR23B and UBQLN2 into aggregates via conjugated ubiquitin

(2018) Hui Yang et al.   FASEB JOURNAL

Multiple clinical features of Huntington’s disease correlate with mutant HTT gene CAG repeat lengths and neurodegeneration

(2018) Sonia Podvin et al.   JOURNAL OF NEUROLOGY

Herp Promotes Degradation of Mutant Huntingtin: Involvement of the Proteasome and Molecular Chaperones

(2018) Huanhuan Luo et al.   MOLECULAR NEUROBIOLOGY

UniProt: the universal protein knowledgebase

(2018) The UniProt Consortium   NUCLEIC ACIDS RESEARCH

Altered Expression of Matrix Metalloproteinases and Their Endogenous Inhibitors in a Human Isogenic Stem Cell Model of Huntington's Disease

(2018) Swati Naphade et al.   Frontiers in Neuroscience

Identification of a splice variant of optineurin which is defective in autophagy and phosphorylation

(2018) Shivranjani C. Moharir et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The ATPase VCP/p97 functions as a disaggregase against toxic Huntingtin-exon1 aggregates

(2018) Debasish Kumar Ghosh et al.   FEBS LETTERS

Mechanism suppressing H3K9 trimethylation in pluripotent stem cells and its demise by polyQ-expanded huntingtin mutations

(2018) Dilber Irmak et al.   HUMAN MOLECULAR GENETICS

Regulatory mechanisms of incomplete huntingtin mRNA splicing

(2018) Andreas Neueder et al.   Nature Communications

Hippo Signaling Pathway Dysregulation in Human Huntington’s Disease Brain and Neuronal Stem Cells

(2018) Kaly A. Mueller et al.   Scientific Reports

High-mobility group box 1 links sensing of reactive oxygen species by huntingtin to its nuclear entry

(2018) Susie Son et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Systematic genetic interaction studies identify histone demethylase Utx as potential target for ameliorating Huntington’s disease

(2017) Wan Song et al.   HUMAN MOLECULAR GENETICS

Polyglutamine tracts regulate beclin 1-dependent autophagy

(2017) Avraham Ashkenazi et al.   NATURE

N-type Ca 2+ channels are affected by full-length mutant huntingtin expression in a mouse model of Huntington's disease

(2017) Flavia R. Silva et al.   NEUROBIOLOGY OF AGING

Structural Basis for Substrate Recognition by the Ankyrin Repeat Domain of Human DHHC17 Palmitoyltransferase

(2017) Raffaello Verardi et al.   STRUCTURE

Cellular toxicity of yeast prion protein Rnq1 can be modulated by N-terminal wild type huntingtin

(2016) Ratnika Sethi et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Biophysical Aspect of Huntingtin Protein During polyQ: An In Silico Insight

(2016) Chandrasekhar Gopalakrishnan et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

Interactome network analysis identifies multiple caspase-6 interactors involved in the pathogenesis of HD

(2016) Sean-Patrick Riechers et al.   HUMAN MOLECULAR GENETICS

Huntingtin-associated protein-1 is a synapsin I-binding protein regulating synaptic vesicle exocytosis and synapsin I trafficking

(2016) Kimberly D. Mackenzie et al.   JOURNAL OF NEUROCHEMISTRY

Downregulation of GABAA Receptor Recycling Mediated by HAP1 Contributes to Neuronal Death in In Vitro Brain Ischemia

(2016) Miranda Mele et al.   MOLECULAR NEUROBIOLOGY

The Biology of Huntingtin

(2016) Frédéric Saudou et al.   NEURON

VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington’s disease

(2016) Xing Guo et al.   Nature Communications

Blocking the association of HDAC4 with MAP1S accelerates autophagy clearance of mutant Huntingtin

(2015) Fei Yue et al.   Aging-US

Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism

(2015) Wen-Chuan Shen et al.   Autophagy

Chaperone protein HYPK interacts with the first 17 amino acid region of Huntingtin and modulates mutant HTT-mediated aggregation and cytotoxicity

(2015) Kamalika Roy Choudhury et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Huntington's disease: An update of therapeutic strategies

(2015) Ashok Kumar et al.   GENE

Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity

(2015) Martin Stroedicke et al.   GENOME RESEARCH

HAP1 helps to regulate actin-based transport of insulin-containing granules in pancreatic β cells

(2015) Zhiyong Wang et al.   HISTOCHEMISTRY AND CELL BIOLOGY

Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Spanin Vivo

(2015) Robert O'Brien et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structural Mechanisms of Mutant Huntingtin Aggregation Suppression by the Synthetic Chaperonin-like CCT5 Complex Explained by Cryoelectron Tomography

(2015) Michele C. Darrow et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Huntingtin-associated Protein 1 (HAP1) Is a cGMP-dependent Kinase Anchoring Protein (GKAP) Specific for the cGMP-dependent Protein Kinase Iβ Isoform

(2015) Eleonora Corradini et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Wnt Signaling Antagonist Dapper1 Accelerates Dishevelled2 Degradation via Promoting Its Ubiquitination and Aggregate-induced Autophagy

(2015) Benyu Ma et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cell and Context-Dependent Effects of the Heat Shock Protein DNAJB6 on Neuronal Survival

(2015) Chad Smith et al.   MOLECULAR NEUROBIOLOGY

ENC1 Modulates the Aggregation and Neurotoxicity of Mutant Huntingtin Through p62 Under ER Stress

(2015) Huikyong Lee et al.   MOLECULAR NEUROBIOLOGY

c-Fos Protects Neurons Through a Noncanonical Mechanism Involving HDAC3 Interaction: Identification of a 21-Amino Acid Fragment with Neuroprotective Activity

(2015) Varun Rawat et al.   MOLECULAR NEUROBIOLOGY

Inhibition of Aggregation of Mutant Huntingtin by Nucleic Acid Aptamers In Vitro and in a Yeast Model of Huntington's Disease

(2015) Rajeev K Chaudhary et al.   MOLECULAR THERAPY

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

SerpinB2 (PAI-2) Modulates Proteostasis via Binding Misfolded Proteins and Promotion of Cytoprotective Inclusion Formation

(2015) Jodi A. Lee et al.   PLoS One

Modulation of mitochondrial function by stem cell-derived cellular components

(2014) Tian Liu et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Roles of Hsp104 and trehalose in solubilisation of mutant huntingtin in heat shocked Saccharomyces cerevisiae cells

(2014) Aliabbas A. Saleh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

The palmitoyl acyltransferase HIP14 shares a high proportion of interactors with huntingtin: implications for a role in the pathogenesis of Huntington's disease

(2014) Stefanie L. Butland et al.   HUMAN MOLECULAR GENETICS

Comparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington's disease

(2014) Brett A. Barbaro et al.   HUMAN MOLECULAR GENETICS

Molecular Interaction between the Chaperone Hsc70 and the N-terminal Flank of Huntingtin Exon 1 Modulates Aggregation

(2014) Elodie Monsellier et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Large Scale Huntingtin Protein Interaction Network Implicates Rho GTPase Signaling Pathways in Huntington Disease

(2014) Cendrine Tourette et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

Profilin-2 increased expression and its altered interaction with β-actin in the striatum of 3-nitropropionic acid-induced Huntington’s disease in rats

(2014) J. Chakraborty et al.   NEUROSCIENCE

Inactivation of Apaf1 reduces the formation of mutant huntingtin-dependent aggregates and cell death

(2014) M. Sancho et al.   NEUROSCIENCE

Mutant huntingtin replaces Gab1 and interacts with C-terminal SH3 domain of growth factor receptor binding protein 2 (Grb2)

(2014) Shounak Baksi et al.   NEUROSCIENCE RESEARCH

Identification of Binding Sites in Huntingtin for the Huntingtin Interacting Proteins HIP14 and HIP14L

(2014) Shaun S. Sanders et al.   PLoS One

Polyglutamine-Rich Suppressors of Huntingtin Toxicity Act Upstream of Hsp70 and Sti1 in Spatial Quality Control of Amyloid-Like Proteins

(2014) Katie J. Wolfe et al.   PLoS One

Differential Effect of HDAC3 on Cytoplasmic and Nuclear Huntingtin Aggregates

(2014) Tatsuo Mano et al.   PLoS One

Potential function for the Huntingtin protein as a scaffold for selective autophagy

(2014) Joseph Ochaba et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Overexpression of Q-rich prion-like proteins suppresses polyQ cytotoxicity and alters the polyQ interactome

(2014) Leslie Ripaud et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Huntingtin promotes mTORC1 signaling in the pathogenesis of Huntington's disease

(2014) W. M. Pryor et al.   Science Signaling

Autoinhibitory Structure of the WW Domain of HYPB/SETD2 Regulates Its Interaction with the Proline-Rich Region of Huntingtin

(2014) Yong-Guang Gao et al.   STRUCTURE

Essential Genetic Interactors of SIR2 Required for Spatial Sequestration and Asymmetrical Inheritance of Protein Aggregates

(2014) Jia Song et al.   PLoS Genetics

Ubiquitin–proteasome system involvement in Huntington’s disease

(2014) Zaira Ortega et al.   Frontiers in Molecular Neuroscience

DNAJB6 is a peptide-binding chaperone which can suppress amyloid fibrillation of polyglutamine peptides at substoichiometric molar ratios

(2013) Cecilia Månsson et al.   CELL STRESS & CHAPERONES

Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis

(2013) C. Y. Daniel Lee et al.   FEBS Journal

MeCP2: a novel Huntingtin interactor

(2013) K. N. McFarland et al.   HUMAN MOLECULAR GENETICS

The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal

(2013) T. Maiuri et al.   HUMAN MOLECULAR GENETICS

Rhes, a Striatal-selective Protein Implicated in Huntington Disease, Binds Beclin-1 and Activates Autophagy

(2013) Robert G. Mealer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Disassociation of Histone Deacetylase-3 from Normal Huntingtin Underlies Mutant Huntingtin Neurotoxicity

(2013) F. H. Bardai et al.   JOURNAL OF NEUROSCIENCE

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

(2013) Kyota Fujita et al.   Nature Communications

Degradation of mutant huntingtin via the ubiquitin/proteasome system is modulated by FE65

(2012) Wan Ning Vanessa Chow et al.   BIOCHEMICAL JOURNAL

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

(2012) John Labbadia et al.   BRAIN

Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis

(2012) Tamara Ratovitski et al.   CELL CYCLE

Mutant huntingtin affects endocytosis in striatal cells by altering the binding of AP-2 to membranes

(2012) Janina E. Borgonovo et al.   EXPERIMENTAL NEUROLOGY

Hip14l-deficient mice develop neuropathological and behavioural features of Huntington disease

(2012) Liza M. Sutton et al.   HUMAN MOLECULAR GENETICS

Proteomic Analysis of Wild-type and Mutant Huntingtin-associated Proteins in Mouse Brains Identifies Unique Interactions and Involvement in Protein Synthesis

(2012) Brady P. Culver et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ROCK-phosphorylated vimentin modifies mutant huntingtin aggregation via sequestration of IRBIT

(2012) Peter O Bauer et al.   Molecular Neurodegeneration

Network Organization of the Huntingtin Proteomic Interactome in Mammalian Brain

(2012) Dyna I. Shirasaki et al.   NEURON

In-vivo evidence for the disruption of Rab11 vesicle transport by loss of huntingtin

(2012) Derek Power et al.   NEUROREPORT

Variation within the Huntington's Disease Gene Influences Normal Brain Structure

(2012) Mark Mühlau et al.   PLoS One

A series of N-terminal epitope tagged Hdh knock-in alleles expressing normal and mutant huntingtin: their application to understanding the effect of increasing the length of normal huntingtin’s polyglutamine stretch on CAG140 mouse model pathogenesis

(2012) Shuqiu Zheng et al.   Molecular Brain

A Genome-Scale RNA–Interference Screen Identifies RRAS Signaling as a Pathologic Feature of Huntington's Disease

(2012) John P. Miller et al.   PLoS Genetics

Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation

(2011) L. S. Havel et al.   HUMAN MOLECULAR GENETICS

Novel mechanism of Hsp70 chaperone-mediated prevention of polyglutamine aggregates in a cellular model of huntington disease

(2011) I. V. Guzhova et al.   HUMAN MOLECULAR GENETICS

Wild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14

(2011) Kun Huang et al.   HUMAN MOLECULAR GENETICS

Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions

(2011) Aurélien Davranche et al.   HUMAN MOLECULAR GENETICS

Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease

(2011) U. P. Shirendeb et al.   HUMAN MOLECULAR GENETICS

Interaction with Polyglutamine-expanded Huntingtin Alters Cellular Distribution and RNA Processing of Huntingtin Yeast Two-hybrid Protein A (HYPA)

(2011) Ya-Jun Jiang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prothymosin-α Interacts with Mutant Huntingtin and Suppresses Its Cytotoxicity in Cell Culture

(2011) Gaofeng Dong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Tumor Necrosis Factor Receptor-associated Factor 6 (TRAF6) Associates with Huntingtin Protein and Promotes Its Atypical Ubiquitination to Enhance Aggregate Formation

(2011) Silvia Zucchelli et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of RE1 Protein Silencing Transcription Factor (REST) Expression by HIP1 Protein Interactor (HIPPI)

(2011) Moumita Datta et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Screen for Enhancers of Clearance Identifies Huntingtin as a Heat Shock Protein 90 (Hsp90) Client Protein

(2011) Barbara Baldo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Optineurin in Huntington's disease intranuclear inclusions

(2011) Claudia Schwab et al.   NEUROSCIENCE LETTERS

Exploring the Correlates of Intermediate CAG Repeats in Huntington Disease

(2011) Ainhi D. Ha et al.   POSTGRADUATE MEDICINE

Ser46 phosphorylation and prolyl-isomerase Pin1-mediated isomerization of p53 are key events in p53-dependent apoptosis induced by mutant huntingtin

(2011) A. Grison et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Evolutionarily Conserved Interaction Between LC3 and p62 Selectively Mediates Autophagy-Dependent Degradation of Mutant Huntingtin

(2010) Ying-Tsen Tung et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

(2010) Christian Landles et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

The Chaperone-Like Protein HYPK Acts Together with NatA in Cotranslational N-Terminal Acetylation and Prevention of Huntingtin Aggregation

(2010) T. Arnesen et al.   MOLECULAR AND CELLULAR BIOLOGY

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

Huntingtin Interacts with the Cue Domain of gp78 and Inhibits gp78 Binding to Ubiquitin and p97/VCP

(2010) Hui Yang et al.   PLoS One

pARIS-htt: an optimised expression platform to study huntingtin reveals functional domains required for vesicular trafficking

(2010) Raúl Pardo et al.   Molecular Brain

Identification of the key structural motifs involved in HspB8/HspB6–Bag3 interaction

(2009) Margit Fuchs et al.   BIOCHEMICAL JOURNAL

Neuroprotective Effects of Calmodulin Peptide 76-121aa: Disruption of Calmodulin Binding to Mutant Huntingtin

(2009) Nichole L. Dudek et al.   BRAIN PATHOLOGY

Mutant huntingtin interacts with  -tubulin and disrupts vesicular transport and insulin secretion

(2009) R. Smith et al.   HUMAN MOLECULAR GENETICS

Huntingtin-associated Protein-1 Interacts with Pro-brain-derived Neurotrophic Factor and Mediates Its Transport and Release

(2009) Linda Lin-yan Wu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Inhibition of Rho Kinases Enhances the Degradation of Mutant Huntingtin

(2009) Peter O. Bauer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Modulation of Polyglutamine Conformations and Dimer Formation by the N-Terminus of Huntingtin

(2009) Tim E. Williamson et al.   JOURNAL OF MOLECULAR BIOLOGY

Striatal Expression of a Calmodulin Fragment Improved Motor Function, Weight Loss, and Neuropathology in the R6/2 Mouse Model of Huntington's Disease

(2009) Y. Dai et al.   JOURNAL OF NEUROSCIENCE

Interaction of Postsynaptic Density Protein-95 with NMDA Receptors Influences Excitotoxicity in the Yeast Artificial Chromosome Mouse Model of Huntington's Disease

(2009) J. Fan et al.   JOURNAL OF NEUROSCIENCE

α Pix enhances mutant huntingtin aggregation

(2009) Makoto Eriguchi et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Inhibition of apoptosis signal-regulating kinase 1 reduces endoplasmic reticulum stress and nuclear huntingtin fragments in a mouse model of Huntington disease

(2009) K.J. Cho et al.   NEUROSCIENCE

ToppGene Suite for gene list enrichment analysis and candidate gene prioritization

(2009) J. Chen et al.   NUCLEIC ACIDS RESEARCH

Detection of Alpha-Rod Protein Repeats Using a Neural Network and Application to Huntingtin

(2009) Gareth A. Palidwor et al.   PLoS Computational Biology

Abnormal proteins can form aggresome in yeast: aggresome-targeting signals and components of the machinery

(2008) Yan Wang et al.   FASEB JOURNAL

Phosphorylation of mutant huntingtin at S421 restores anterograde and retrograde transport in neurons

(2008) Diana Zala et al.   HUMAN MOLECULAR GENETICS

Actin Binding by Hip1 (Huntingtin-interacting Protein 1) and Hip1R (Hip1-related Protein) Is Regulated by Clathrin Light Chain

(2008) Jeremy D. Wilbur et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Huntingtin Regulates RE1-silencing Transcription Factor/Neuron-restrictive Silencer Factor (REST/NRSF) Nuclear Trafficking Indirectly through a Complex with REST/NRSF-interacting LIM Domain Protein (RILP) and Dynactin p150Glued

(2008) Masahito Shimojo   JOURNAL OF BIOLOGICAL CHEMISTRY

Protective Effects of Interrupting the Binding of Calmodulin to Mutant Huntingtin

(2008) Nichole L. Dudek et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Suppression of Mutant Huntingtin Aggregate Formation by Cdk5/p35 through the Effect on Microtubule Stability

(2008) S. Kaminosono et al.   JOURNAL OF NEUROSCIENCE

Altered Histone Monoubiquitylation Mediated by Mutant Huntingtin Induces Transcriptional Dysregulation

(2008) M.-O. Kim et al.   JOURNAL OF NEUROSCIENCE

Phosphorylation of Profilin by ROCK1 Regulates Polyglutamine Aggregation

(2008) J. Shao et al.   MOLECULAR AND CELLULAR BIOLOGY

Functional roles for the striatal-enriched transcription factor, Bcl11b, in the control of striatal gene expression and transcriptional dysregulation in Huntington's disease

(2008) Paula A. Desplats et al.   NEUROBIOLOGY OF DISEASE