N-alpha-acetylation of Huntingtin protein increases its propensity to aggregate

Flanking Regions Determine the Structure of the Poly-Glutamine in Huntingtin through Mechanisms Common among Glutamine-Rich Human Proteins

(2020) Annika Urbanek et al.   STRUCTURE

Truncation of mutant huntingtin in knock-in mice demonstrates exon1 huntingtin is a key pathogenic form

(2020) Huiming Yang et al.   Nature Communications

Protofilament Structure and Supramolecular Polymorphism of Aggregated Mutant Huntingtin Exon 1

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

Protein N-Terminal Acetylation: Structural Basis, Mechanism, Versatility, and Regulation

(2020) Sunbin Deng et al.   TRENDS IN BIOCHEMICAL SCIENCES

Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis

(2020) Ramita Dewan et al.   NEURON

Dual Role of Ribosome-Binding Domain of NAC as a Potent Suppressor of Protein Aggregation and Aging-Related Proteinopathies

(2019) Koning Shen et al.   MOLECULAR CELL

Clinical phenotype in carriers of intermediate alleles in the huntingtin gene

(2019) Daniel Savitt et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Identification of N-linked glycans as specific mediators of neuronal uptake of acetylated α-Synuclein

(2019) Melissa Birol et al.   PLOS BIOLOGY

Nucleation Inhibition of Huntingtin Protein (htt) by Polyproline PPII Helices: A Potential Interaction with the N-Terminal α-Helical Region of Htt

(2019) James R. Arndt et al.   BIOCHEMISTRY

Disordered Nanostructure in Huntingtin Interacting Protein K Acts as a Stabilizing Switch To Prevent Protein Aggregation

(2018) Debasish Kumar Ghosh et al.   BIOCHEMISTRY

Self-assembly of Mutant Huntingtin Exon-1 Fragments into Large Complex Fibrillar Structures Involves Nucleated Branching

(2018) Anne S. Wagner et al.   JOURNAL OF MOLECULAR BIOLOGY

Structure of Human NatA and Its Regulation by the Huntingtin Interacting Protein HYPK

(2018) Leah Gottlieb et al.   STRUCTURE

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

Mutational analysis implicates the amyloid fibril as the toxic entity in Huntington's disease

(2018) Kenneth W. Drombosky et al.   NEUROBIOLOGY OF DISEASE

Role of Parkinson’s Disease-Linked Mutations and N-Terminal Acetylation on the Oligomerization of α-Synuclein Induced by 3,4-Dihydroxyphenylacetaldehyde

(2018) Vanderlei de Araújo Lima et al.   ACS Chemical Neuroscience

The 17-residue-long N terminus in huntingtin controls stepwise aggregation in solution and on membranes via different mechanisms

(2017) Nitin K. Pandey et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington’s disease

(2017) Rocio Gomez-Pastor et al.   Nature Communications

Assembly of Huntingtin headpiece into α-helical bundles

(2017) Beytullah Ozgur et al.   Biointerphases

Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch

(2017) E. F. E. Kuiper et al.   Frontiers in Neuroscience

HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models

(2017) Andreas Neueder et al.   Scientific Reports

Acetylation within the First 17 Residues of Huntingtin Exon 1 Alters Aggregation and Lipid Binding

(2016) Maxmore Chaibva et al.   BIOPHYSICAL JOURNAL

Differential proteomic and genomic profiling of mouse striatal cell model of Huntington's disease and control; probable implications to the disease biology

(2016) Kamalika Roy Choudhury et al.   Journal of Proteomics

Late onset Huntington's disease with 29 CAG repeat expansion

(2016) Pedro J. Garcia-Ruiz et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Reply: Late onset Huntington's disease with 29 CAG repeat expansion

(2016) Mayke Oosterloo et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

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

(2016) Koning Shen et al.   eLife

Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer

(2016) Bankanidhi Sahoo et al.   PLoS One

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

Detection of ubiquitinated huntingtin species in intracellular aggregates

(2015) Katrin Juenemann et al.   Frontiers in Molecular Neuroscience

Aged monkey brains reveal the role of ubiquitin-conjugating enzyme UBE2N in the synaptosomal accumulation of mutant huntingtin

(2014) Peng Yin 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

Conserved C-terminal nascent peptide binding domain of HYPK facilitates its chaperone-like activity

(2014) Swasti Raychaudhuri et al.   JOURNAL OF BIOSCIENCES

Polyglutamine Aggregation in Huntington Disease: Does Structure Determine Toxicity?

(2014) Guylaine Hoffner et al.   MOLECULAR NEUROBIOLOGY

N-Alpha-Acetylation of α-Synuclein Increases Its Helical Folding Propensity, GM1 Binding Specificity and Resistance to Aggregation

(2014) Tim Bartels et al.   PLoS One

Loss of amino-terminal acetylation suppresses a prion phenotype by modulating global protein folding

(2014) William M. Holmes et al.   Nature Communications

Membrane Interactions of the Amphipathic Amino Terminus of Huntingtin

(2013) Matthias Michalek et al.   BIOCHEMISTRY

Heat shock transcription factor HSF1 regulates the expression of the Huntingtin-interacting protein HYPK

(2013) Hiroshi Sakurai et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Structure and Topology of the Huntingtin 1–17 Membrane Anchor by a Combined Solution and Solid-State NMR Approach

(2013) Matthias Michalek et al.   BIOPHYSICAL JOURNAL

Exploring the accessible conformations of N-terminal acetylated α-synuclein

(2013) Gina M. Moriarty et al.   FEBS LETTERS

N-terminal Acetylation Stabilizes N-terminal Helicity in Lipid- and Micelle-bound α-Synuclein and Increases Its Affinity for Physiological Membranes

(2013) Igor Dikiy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

β-Hairpin-Mediated Nucleation of Polyglutamine Amyloid Formation

(2013) Karunakar Kar et al.   JOURNAL OF MOLECULAR BIOLOGY

Temporal Separation of Aggregation and Ubiquitination during Early Inclusion Formation in Transgenic Mice Carrying the Huntington’s Disease Mutation

(2012) Belvin Gong et al.   PLoS One

Identification of HYPK-Interacting Proteins Reveals Involvement of HYPK in Regulating Cell Growth, Cell Cycle, Unfolded Protein Response and Cell Death

(2012) Kamalika Roy Choudhury et al.   PLoS One

N-terminal acetylation of α-synuclein induces increased transient helical propensity and decreased aggregation rates in the intrinsically disordered monomer

(2012) Lijuan Kang et al.   PROTEIN SCIENCE

Structure of a Ternary Naa50p (NAT5/SAN) N-terminal Acetyltransferase Complex Reveals the Molecular Basis for Substrate-specific Acetylation

(2011) Glen Liszczak et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

α-Synuclein occurs physiologically as a helically folded tetramer that resists aggregation

(2011) Tim Bartels et al.   NATURE

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

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

NatF Contributes to an Evolutionary Shift in Protein N-Terminal Acetylation and Is Important for Normal Chromosome Segregation

(2011) Petra Van Damme et al.   PLoS Genetics

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

Acetylation Targets Mutant Huntingtin to Autophagosomes for Degradation

(2009) Hyunkyung Jeong et al.   CELL

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

Proteomics analyses reveal the evolutionary conservation and divergence of N-terminal acetyltransferases from yeast and humans

(2009) T. Arnesen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The NatA Acetyltransferase Couples Sup35 Prion Complexes to the [PSI+] Phenotype

(2008) John A. Pezza et al.   MOLECULAR BIOLOGY OF THE CELL

Re: Autopsy-proven Huntington's disease with 29 trinucleotide repeats

(2008) Alicia Semaka et al.   MOVEMENT DISORDERS