Polyglutamine Aggregation in Huntington Disease: Does Structure Determine Toxicity?

Molecular Structures of Amyloid and Prion Fibrils: Consensus versus Controversy

(2013) Robert Tycko et al.   ACCOUNTS OF CHEMICAL RESEARCH

Structure of Inclusions of Huntington’s Disease Brain Revealed by Synchrotron Infrared Microspectroscopy: Polymorphism and Relevance to Cytotoxicity

(2013) William André et al.   ANALYTICAL CHEMISTRY

Pharmacological protein targets in polyglutamine diseases: Mutant polypeptides and their interactors

(2013) Boris A. Margulis et al.   FEBS LETTERS

Linear and extended: a common polyglutamine conformation recognized by the three antibodies MW1, 1C2 and 3B5H10

(2013) Fabrice A. C. Klein et al.   HUMAN MOLECULAR GENETICS

Inhibition of Protein Misfolding/Aggregation Using Polyglutamine Binding Peptide QBP1 as a Therapy for the Polyglutamine Diseases

(2013) H. Akiko Popiel et al.   Neurotherapeutics

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

(2013) K. Sathasivam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis

(2012) Jean D. Sipe et al.   AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS

Induced Pluripotent Stem Cells from Patients with Huntington's Disease Show CAG-Repeat-Expansion-Associated Phenotypes

(2012) The HD iPSC Consortium   Cell Stem Cell

TR-FRET-Based Duplex Immunoassay Reveals an Inverse Correlation of Soluble and Aggregated Mutant huntingtin in Huntington's Disease

(2012) Barbara Baldo et al.   CHEMISTRY & BIOLOGY

Transglutaminase-Catalyzed Crosslinking in Neurological Disease: From Experimental Evidence to Therapeutic Inhibition

(2012) G. Hoffner et al.   CNS & Neurological Disorders-Drug Targets

Identification of Novel Potentially Toxic Oligomers Formedin Vitrofrom Mammalian-derived Expanded huntingtin Exon-1 Protein

(2012) Leslie G. Nucifora et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Disease-Associated Polyglutamine Stretches in Monomeric Huntingtin Adopt a Compact Structure

(2012) Clare Peters-Libeu et al.   JOURNAL OF MOLECULAR BIOLOGY

Serine Phosphorylation Suppresses Huntingtin Amyloid Accumulation by Altering Protein Aggregation Properties

(2012) Rakesh Mishra et al.   JOURNAL OF MOLECULAR BIOLOGY

Physical Chemistry of Polyglutamine: Intriguing Tales of a Monotonous Sequence

(2012) Ronald Wetzel   JOURNAL OF MOLECULAR BIOLOGY

The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington's disease patients demonstrates mutation related enhanced lysosomal activity

(2012) Stefano Camnasio et al.   NEUROBIOLOGY OF DISEASE

Fragments of HdhQ150 Mutant Huntingtin Form a Soluble Oligomer Pool That Declines with Aggregate Deposition upon Aging

(2012) David Marcellin et al.   PLoS One

Antiparallel  -sheet architecture in Iowa-mutant  -amyloid fibrils

(2012) W. Qiang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington’s disease patient cells

(2012) Tarja A Juopperi et al.   Molecular Brain

The Amyloid-Congo Red Interface at Atomic Resolution

(2011) Anne K. Schütz et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Aging causes distinct characteristics of polyglutamine amyloids in vivo

(2011) Ayako Tonoki et al.   GENES TO CELLS

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

Protein Misfolding Detected Early in Pathogenesis of Transgenic Mouse Model of Huntington Disease Using Amyloid Seeding Assay

(2011) Sharad Gupta et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Compact β Model of huntingtin Toxicity

(2011) Qi Charles Zhang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structural Characterization of Polyglutamine Fibrils by Solid-State NMR Spectroscopy

(2011) Robert Schneider et al.   JOURNAL OF MOLECULAR BIOLOGY

Inhibiting the Nucleation of Amyloid Structure in a Huntingtin Fragment by Targeting α-Helix-Rich Oligomeric Intermediates

(2011) Rakesh Mishra et al.   JOURNAL OF MOLECULAR BIOLOGY

Slow Amyloid Nucleation via α-Helix-Rich Oligomeric Intermediates in Short Polyglutamine-Containing Huntingtin Fragments

(2011) Murali Jayaraman et al.   JOURNAL OF MOLECULAR BIOLOGY

The Aggregation-Enhancing Huntingtin N-Terminus Is Helical in Amyloid Fibrils

(2011) V. N. Sivanandam et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Kinase inhibitors modulate huntingtin cell localization and toxicity

(2011) Randy Singh Atwal et al.   Nature Chemical Biology

Critical nucleus size for disease-related polyglutamine aggregation is repeat-length dependent

(2011) Karunakar Kar et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Induced pluripotent stem cell lines from Huntington's disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway

(2011) Valentina Castiglioni et al.   NEUROBIOLOGY OF DISEASE

Small Changes, Big Impact

(2011) Dagmar E. Ehrnhoefer et al.   NEUROSCIENTIST

Perturbation with Intrabodies Reveals That Calpain Cleavage Is Required for Degradation of Huntingtin Exon 1

(2011) Amber L. Southwell et al.   PLoS One

Essential Role of Coiled Coils for Aggregation and Activity of Q/N-Rich Prions and PolyQ Proteins

(2010) Ferdinando Fiumara et al.   CELL

Reprogramming Huntington Monkey Skin Cells into Pluripotent Stem Cells

(2010) Anthony W.S. Chan et al.   Cellular Reprogramming

Mutant Huntingtin Fragments Form Oligomers in a Polyglutamine Length-dependent Mannerin Vitroandin Vivo

(2010) Justin Legleiter et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Tracking Mutant Huntingtin Aggregation Kinetics in Cells Reveals Three Major Populations That Include an Invariant Oligomer Pool

(2010) Maya A. Olshina et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Polyglutamine Induced Misfolding of Huntingtin Exon1 is Modulated by the Flanking Sequences

(2010) Vinal V. Lakhani et al.   PLoS Computational Biology

Mechanism of Cis-Inhibition of PolyQ Fibrillation by PolyP: PPII Oligomers and the Hydrophobic Effect

(2009) Gregory D. Darnell et al.   BIOPHYSICAL JOURNAL

Post-translational modifications of expanded polyglutamine proteins: impact on neurotoxicity

(2009) M. Pennuto et al.   HUMAN MOLECULAR GENETICS

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease

(2009) Kirupa Sathasivam et al.   HUMAN MOLECULAR GENETICS

Mutant Huntingtin N-terminal Fragments of Specific Size Mediate Aggregation and Toxicity in Neuronal Cells

(2009) Tamara Ratovitski et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Monoclonal Antibodies Recognize Distinct Conformational Epitopes Formed by Polyglutamine in a Mutant Huntingtin Fragment

(2009) Justin Legleiter 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

Cross-Seeding Fibrillation of Q/N-Rich Proteins Offers New Pathomechanism of Polyglutamine Diseases

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

Protein folding: Sticky N17 speeds huntingtin pile-up

(2009) Susan W Liebman et al.   Nature Chemical 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

Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity

(2009) Y. Nekooki-Machida et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Secondary Structure of Huntingtin Amino-Terminal Region

(2009) Mee Whi Kim et al.   STRUCTURE

Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease

(2008) Chuan-En Wang et al.   HUMAN MOLECULAR GENETICS

Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice

(2008) M. Gray et al.   JOURNAL OF NEUROSCIENCE

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice

(2008) A Jennifer Morton et al.   NEUROBIOLOGY OF DISEASE

CAG repeat lengths ≥335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse

(2008) I. Dragatsis et al.   NEUROBIOLOGY OF DISEASE

FTIR reveals structural differences between native β-sheet proteins and amyloid fibrils

(2004) Giorgia Zandomeneghi et al.   PROTEIN SCIENCE