Nuclear and cytoplasmic huntingtin inclusions exhibit distinct biochemical composition, interactome and ultrastructural properties

The Nt17 domain and its helical conformation regulate the aggregation, cellular properties and neurotoxicity of mutant huntingtin exon 1

(2021) Sophie Vieweg et al.   JOURNAL OF MOLECULAR BIOLOGY

A New Chemoenzymatic Semisynthetic Approach Provides Insight into the Role of Phosphorylation beyond Exon1 of Huntingtin and Reveals N-Terminal Fragment Length-Dependent Distinct Mechanisms of Aggregation

(2021) Rajasekhar Kolla et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Effects of flanking sequences and cellular context on subcellular behavior and pathology of mutant HTT

(2020) Anjalika Chongtham et al.   HUMAN MOLECULAR GENETICS

The process of Lewy body formation, rather than simply α-synuclein fibrillization, is one of the major drivers of neurodegeneration

(2020) Anne-Laure Mahul-Mellier et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Live-Cell Imaging and Quantification of PolyQ Aggregates by Stimulated Raman Scattering of Selective Deuterium Labeling

(2020) Kun Miao et al.   ACS Central Science

Mitochondrial fragmentation enables localized signaling required for cell repair

(2020) Adam Horn et al.   JOURNAL OF CELL BIOLOGY

Robust summarization and inference in proteome-wide label-free quantification

(2020) Adriaan Sticker et al.   MOLECULAR & CELLULAR PROTEOMICS

Protofilament Structure and Supramolecular Polymorphism of Aggregated Mutant Huntingtin Exon 1

(2020) Jennifer C. Boatz et al.   JOURNAL OF MOLECULAR BIOLOGY

Investigating the Transition of Pre-Symptomatic to Symptomatic Huntington’s Disease Status Based on Omics Data

(2020) Christiana C. Christodoulou et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Global Proteome and Ubiquitinome Changes in the Soluble and Insoluble Fractions of Q175 Huntington Mice Brains

(2019) Karen A. Sap et al.   MOLECULAR & CELLULAR PROTEOMICS

Lewy pathology in Parkinson’s disease consists of crowded organelles and lipid membranes

(2019) Sarah H. Shahmoradian et al.   NATURE NEUROSCIENCE

Mutant huntingtin interacts with the sterol regulatory element-binding proteins and impairs their nuclear import

(2019) Alba Di Pardo et al.   HUMAN MOLECULAR GENETICS

Profilin reduces aggregation and phase separation of huntingtin N-terminal fragments by preferentially binding to soluble monomers and oligomers

(2018) Ammon E. Posey et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Establishment of Constraints on Amyloid Formation Imposed by Steric Exclusion of Globular Domains

(2018) Rafayel A. Azizyan et al.   JOURNAL OF MOLECULAR BIOLOGY

A Liquid to Solid Phase Transition Underlying Pathological Huntingtin Exon1 Aggregation

(2018) Thomas R. Peskett et al.   MOLECULAR CELL

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

(2018) Huanhuan Luo et al.   MOLECULAR NEUROBIOLOGY

Molecular and structural architecture of polyQ aggregates in yeast

(2018) Anselm Gruber et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The S1P Axis: New Exciting Route for Treating Huntington’s Disease

(2018) Alba Di Pardo et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

Dynamic recruitment of ubiquitin to mutant huntingtin inclusion bodies

(2018) Katrin Juenemann et al.   Scientific Reports

N-terminal Huntingtin (Htt) phosphorylation is a molecular switch regulating Htt aggregation, helical conformation, internalization, and nuclear targeting

(2018) Sean M. Deguire et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A role for autophagy in Huntington's disease

(2018) Katherine R. Croce et al.   NEUROBIOLOGY OF DISEASE

Altered ER–mitochondria contact impacts mitochondria calcium homeostasis and contributes to neurodegeneration in vivo in disease models

(2018) Kyu-Sun Lee et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly

(2018) Meng Lu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

NEDDylation promotes nuclear protein aggregation and protects the Ubiquitin Proteasome System upon proteotoxic stress

(2018) Chantal M. Maghames et al.   Nature Communications

Convolutional neural networks for automated annotation of cellular cryo-electron tomograms

(2017) Muyuan Chen et al.   NATURE METHODS

Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport

(2017) Fatima Gasset-Rosa et al.   NEURON

Autophagy Receptors and Neurodegenerative Diseases

(2017) Zhiqiang Deng et al.   TRENDS IN CELL BIOLOGY

Spatiotemporal Proteomic Profiling of Huntington’s Disease Inclusions Reveals Widespread Loss of Protein Function

(2017) Fabian Hosp et al.   Cell Reports

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

(2016) Roland Hjerpe et al.   CELL

Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease

(2016) Xiangling Yin et al.   HUMAN MOLECULAR GENETICS

An 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

(2016) Sophie Vieweg et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Dysfunctional Calcium and Glutamate Signaling in Striatal Astrocytes from Huntington's Disease Model Mice

(2016) R. Jiang et al.   JOURNAL OF NEUROSCIENCE

Soluble Oligomers of PolyQ-Expanded Huntingtin Target a Multiplicity of Key Cellular Factors

(2016) Yujin E. Kim et al.   MOLECULAR CELL

The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity

(2016) Zih-Ning Huang et al.   MOLECULAR NEUROBIOLOGY

The Perseus computational platform for comprehensive analysis of (prote)omics data

(2016) Stefka Tyanova et al.   NATURE METHODS

TFG Promotes Organization of Transitional ER and Efficient Collagen Secretion

(2016) Janine McCaughey et al.   Cell Reports

Real-time imaging of Huntingtin aggregates diverting target search and gene transcription

(2016) Li Li et al.   eLife

Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract

(2016) Koning Shen et al.   eLife

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

Signature changes in ubiquilin expression in the R6/2 mouse model of Huntington’s disease

(2015) Nathaniel Safren et al.   BRAIN RESEARCH

Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation

(2015) M.-T. El-Daher et al.   EMBO JOURNAL

Huntington’s disease biomarker progression profile identified by transcriptome sequencing in peripheral blood

(2015) Anastasios Mastrokolias et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease

(2015) Maria Manczak et al.   HUMAN MOLECULAR GENETICS

Alteration of Lysosome Fusion and Low-grade Inflammation Mediated by Super-low-dose Endotoxin

(2015) Bianca Baker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Differences in mitochondrial function in homogenated samples from healthy and epileptic specific brain tissues revealed by high-resolution respirometry

(2015) Johannes Burtscher et al.   MITOCHONDRION

Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates

(2015) Maggie P. Wear et al.   PLoS One

Malaria Parasite-Infected Erythrocytes Secrete PfCK1, the Plasmodium Homologue of the Pleiotropic Protein Kinase Casein Kinase 1

(2015) Dominique Dorin-Semblat et al.   PLoS One

One-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

(2014) Annalisa Ansaloni et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Aggregation Behavior of Chemically Synthesized, Full-Length Huntingtin Exon1

(2014) Bankanidhi Sahoo et al.   BIOCHEMISTRY

Autophagic Clearance of PolyQ Proteins Mediated by Ubiquitin-Atg8 Adaptors of the Conserved CUET Protein Family

(2014) Kefeng Lu et al.   CELL

c-Abl phosphorylates α-synuclein and regulates its degradation: implication for α-synuclein clearance and contribution to the pathogenesis of Parkinson's disease

(2014) Anne-Laure Mahul-Mellier et al.   HUMAN MOLECULAR GENETICS

Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease

(2014) Kuan-Yu Liu 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

Accurate Proteome-wide Label-free Quantification by Delayed Normalization and Maximal Peptide Ratio Extraction, Termed MaxLFQ

(2014) Jürgen Cox et al.   MOLECULAR & CELLULAR PROTEOMICS

Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors

(2014) Andrea Crotti et al.   NATURE NEUROSCIENCE

Huntington disease: natural history, biomarkers and prospects for therapeutics

(2014) Christopher A. Ross et al.   Nature Reviews Neurology

Ubiquilin-1 Overexpression Increases the Lifespan and Delays Accumulation of Huntingtin Aggregates in the R6/2 Mouse Model of Huntington's Disease

(2014) Nathaniel Safren et al.   PLoS One

Membrane Interactions of the Amphipathic Amino Terminus of Huntingtin

(2013) Matthias Michalek et al.   BIOCHEMISTRY

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies

(2013) Sabine Schipper-Krom et al.   FEBS LETTERS

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

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

An N-terminal Nuclear Export Signal Regulates Trafficking and Aggregation of Huntingtin (Htt) Protein Exon 1

(2013) Zhiqiang Zheng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Interaction of Polyglutamine Peptides with Lipid Membranes Is Regulated by Flanking Sequences Associated with Huntingtin

(2013) Kathleen A. Burke et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Immunohistochemical analysis of ubiquilin-1 in the human hippocampus: Association with neurofibrillary tangle pathology

(2013) Katsuyoshi Mizukami et al.   NEUROPATHOLOGY

Huntington's disease: underlying molecular mechanisms and emerging concepts

(2013) John Labbadia et al.   TRENDS IN BIOCHEMICAL SCIENCES

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

(2013) Julia Leitman et al.   Nature Communications

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

(2012) Tamara Ratovitski et al.   CELL CYCLE

Reactive oxygen species (ROS) homeostasis and redox regulation in cellular signaling

(2012) Paul D. Ray et al.   CELLULAR SIGNALLING

Optineurin and amyotrophic lateral sclerosis

(2012) Hirofumi Maruyama et al.   Geriatrics & Gerontology International

Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells

(2012) Maxime Feyeux et al.   HUMAN MOLECULAR GENETICS

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington’s disease

(2012) Mark S. Hipp et al.   JOURNAL OF CELL BIOLOGY

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion

(2012) J.- M. Lee et al.   NEUROLOGY

Converging Pathways in the Occurrence of Endoplasmic Reticulum (ER) Stress in Huntingtons Disease

(2011) R. Vidal et al.   CURRENT MOLECULAR MEDICINE

TFG-1 function in protein secretion and oncogenesis

(2011) Kristen Witte et al.   NATURE CELL BIOLOGY

Flipping a switch on huntingtin

(2011) Erin R Greiner et al.   Nature Chemical Biology

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

(2011) Wenjun Song et al.   NATURE MEDICINE

Protective role of the ubiquitin binding protein Tollip against the toxicity of polyglutamine-expansion proteins

(2011) Asami Oguro et al.   NEUROSCIENCE LETTERS

Optineurin in Huntington's disease intranuclear inclusions

(2011) Claudia Schwab et al.   NEUROSCIENCE LETTERS

Quantitative proteomics combined with BAC TransgeneOmics reveals in vivo protein interactions

(2010) Nina C. Hubner et al.   JOURNAL OF CELL BIOLOGY

Altered cholesterol homeostasis contributes to enhanced excitotoxicity in Huntington’s disease

(2010) Daniel Del Toro et al.   JOURNAL OF NEUROCHEMISTRY

Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

(2010) J. Miller et al.   JOURNAL OF NEUROSCIENCE

Mechanism of ER Stress-Induced Brain Damage by IP3 Receptor

(2010) Takayasu Higo et al.   NEURON

Neurocognitive signs in prodromal Huntington disease.

(2010) Julie C. Stout et al.   NEUROPSYCHOLOGY

Ubiquilins accelerate autophagosome maturation and promote cell survival during nutrient starvation

(2009) Elsa-Noah N’Diaye et al.   Autophagy

Phosphorylation of Threonine 3

(2009) Charity T. Aiken et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

(2009) Leslie Michels Thompson et al.   JOURNAL OF CELL BIOLOGY

The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation

(2009) Stephen Tam et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism

(2009) Ashwani K Thakur et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Serines 13 and 16 Are Critical Determinants of Full-Length Human Mutant Huntingtin Induced Disease Pathogenesis in HD Mice

(2009) Xiaofeng Gu et al.   NEURON

Adaptation of endoplasmic reticulum exit sites to acute and chronic increases in cargo load

(2008) Hesso Farhan et al.   EMBO JOURNAL

MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification

(2008) Jürgen Cox et al.   NATURE BIOTECHNOLOGY

Mutant huntingtin and mitochondrial dysfunction

(2008) Ella Bossy-Wetzel et al.   TRENDS IN NEUROSCIENCES

Brain gene expression correlates with changes in behavior in the R6/1 mouse model of Huntington’s disease

(2007) A. Hodges et al.   GENES BRAIN AND BEHAVIOR