Nucleolar stress controls mutant Huntington toxicity and monitors Huntington’s disease progression

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

(2021) Mehdi Eshraghi et al.   Nature Communications

Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

(2021) Olubankole Aladesuyi Arogundade et al.   Acta Neuropathologica Communications

The nucleolus as a multiphase liquid condensate

(2020) Denis L. J. Lafontaine et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Phase-to-Phase With Nucleoli – Stress Responses, Protein Aggregation and Novel Roles of RNA

(2019) Leena Latonen   Frontiers in Cellular Neuroscience

Increased translation as a novel pathogenic mechanism in Huntington’s disease

(2019) Jordi Creus-Muncunill et al.   BRAIN

Quality Control in Huntington’s Disease: a Therapeutic Target

(2019) Sachchida Nand Rai et al.   NEUROTOXICITY RESEARCH

The nucleolus functions as a phase-separated protein quality control compartment

(2019) F. Frottin et al.   SCIENCE

CAG Repeat Not Polyglutamine Length Determines Timing of Huntington’s Disease Onset

(2019) Jong-Min Lee et al.   CELL

Nascent Pre-rRNA Sorting via Phase Separation Drives the Assembly of Dense Fibrillar Components in the Human Nucleolus

(2019) Run-Wen Yao et al.   MOLECULAR CELL

The Nucleolus as a Proteostasis Regulator

(2019) Fatima Amer-Sarsour et al.   TRENDS IN CELL BIOLOGY

A role of cellular translation regulation associated with toxic Huntingtin protein

(2019) Hiranmay Joag et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Nucleolus: A Protein Quality Control Compartment

(2019) Pavani Rekulapally et al.   TRENDS IN BIOCHEMICAL SCIENCES

Regulation of RNA Polymerase I TranscriptioninDevelopment, Disease, and Aging

(2018) Samim Sharifi et al.   Annual Review of Biochemistry

New Roles for the Nucleolus in Health and Disease

(2018) Lorena Núñez Villacís et al.   BIOESSAYS

C9orf72-associated neurodegeneration in ALS-FTD: breaking new ground in ribosomal RNA and nucleolar dysfunction

(2018) Dustin Herrmann et al.   CELL AND TISSUE RESEARCH

A Liquid to Solid Phase Transition Underlying Pathological Huntingtin Exon1 Aggregation

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

Use of the iNo score to discriminate normal from altered nucleolar morphology, with applications in basic cell biology and potential in human disease diagnostics

(2018) Vassiliki Stamatopoulou et al.   Nature Protocols

Therapeutic approaches to Huntington disease: from the bench to the clinic

(2018) Nicholas S. Caron et al.   NATURE REVIEWS DRUG DISCOVERY

Nucleolar fibrillarin is an evolutionarily conserved regulator of bacterial pathogen resistance

(2018) Varnesh Tiku et al.   Nature Communications

Mitochondrial cristae remodelling is associated with disrupted OPA1 oligomerisation in the Huntington's disease R6/2 fragment model

(2017) Tanja Hering et al.   EXPERIMENTAL NEUROLOGY

Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease

(2017) Sarah Jesse et al.   Scientific Reports

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

(2016) Kyung-Ha Lee et al.   CELL

Transcriptome sequencing reveals aberrant alternative splicing in Huntington's disease

(2016) Lan Lin et al.   HUMAN MOLECULAR GENETICS

Requirement of Neuronal Ribosome Synthesis for Growth and Maintenance of the Dendritic Tree

(2016) Lukasz P. Slomnicki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of Neuronal Survival by Nucleophosmin 1 (NPM1) Is Dependent on Its Expression Level, Subcellular Localization, and Oligomerization Status

(2016) Jason A. Pfister et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Activating transcription factor 6 derepression mediates neuroprotection in Huntington disease

(2016) José R. Naranjo et al.   JOURNAL OF CLINICAL INVESTIGATION

Integrated genomics and proteomics define huntingtin CAG length–dependent networks in mice

(2016) Peter Langfelder et al.   NATURE NEUROSCIENCE

The Biology of Huntingtin

(2016) Frédéric Saudou et al.   NEURON

Involvement of human ribosomal proteins in nucleolar structure and p53-dependent nucleolar stress

(2016) Emilien Nicolas et al.   Nature Communications

Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing

(2015) Martin H. Schludi et al.   ACTA NEUROPATHOLOGICA

Selective vulnerability of Rich Club brain regions is an organizational principle of structural connectivity loss in Huntington’s disease

(2015) Peter McColgan et al.   BRAIN

Selective striatal mtDNA depletion in end-stage Huntington's disease R6/2 mice

(2015) Tanja Hering et al.   EXPERIMENTAL NEUROLOGY

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

The nucleolar protein nucleophosmin is essential for autophagy induced by inhibiting Pol I transcription

(2015) Naohiro Katagiri et al.   Scientific Reports

Huntington disease

(2015) Gillian P. Bates et al.   Nature Reviews Disease Primers

Characterization of HTT Inclusion Size, Location, and Timing in the zQ175 Mouse Model of Huntington´s Disease: An In Vivo High-Content Imaging Study

(2015) Nikisha Carty et al.   PLoS One

Ribosomal Protein s15 Phosphorylation Mediates LRRK2 Neurodegeneration in Parkinson’s Disease

(2014) Ian Martin et al.   CELL

Nucleolar dysfunction in Huntington's disease

(2013) Junghee Lee et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A neuroprotective phase precedes striatal degeneration upon nucleolar stress

(2013) G Kreiner et al.   CELL DEATH AND DIFFERENTIATION

Expression of Expanded CAG Transcripts Triggers Nucleolar Stress in Huntington's Disease

(2013) Ho Tsoi et al.   CEREBELLUM

The Complexity of Human Ribosome Biogenesis Revealed by Systematic Nucleolar Screening of Pre-rRNA Processing Factors

(2013) Lionel Tafforeau et al.   MOLECULAR CELL

Impaired rRNA synthesis triggers homeostatic responses in hippocampal neurons

(2013) Anna Kiryk et al.   Frontiers in Cellular Neuroscience

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

Nucleolar activity in neurodegenerative diseases: a missing piece of the puzzle?

(2012) Rosanna Parlato et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Fiji: an open-source platform for biological-image analysis

(2012) Johannes Schindelin et al.   NATURE METHODS

Network Organization of the Huntingtin Proteomic Interactome in Mammalian Brain

(2012) Dyna I. Shirasaki et al.   NEURON

Interaction of nucleolin with ribosomal RNA genes and its role in RNA polymerase I transcription

(2012) Rong Cong et al.   NUCLEIC ACIDS RESEARCH

Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175

(2012) Liliana B. Menalled et al.   PLoS One

CAG expansion induces nucleolar stress in polyglutamine diseases

(2012) H. Tsoi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Dysregulation of upstream binding factor-1 acetylation at K352 is linked to impaired ribosomal DNA transcription in Huntington's disease

(2011) J Lee et al.   CELL DEATH AND DIFFERENTIATION

The RNA Polymerase I Transcription Machinery: An Emerging Target for the Treatment of Cancer

(2010) Denis Drygin et al.   Annual Review of Pharmacology and Toxicology

Functional Gene Expression Profiling in Yeast Implicates Translational Dysfunction in Mutant Huntingtin Toxicity

(2010) Eran Tauber et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

RNA Polymerase 1-driven Transcription as a Mediator of BDNF-induced Neurite Outgrowth

(2010) Cynthia Gomes et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

BDNF Overexpression in the Forebrain Rescues Huntington's Disease Phenotypes in YAC128 Mice

(2010) Y. Xie et al.   JOURNAL OF NEUROSCIENCE

The Nucleolus under Stress

(2010) Séverine Boulon et al.   MOLECULAR CELL

What the nucleolus says to a tumour pathologist

(2009) Massimo Derenzini et al.   HISTOPATHOLOGY

Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data

(2009) Sarah J Tabrizi et al.   LANCET NEUROLOGY