Possible Co-Evolution of Polyglutamine and Polyproline in Huntingtin Protein: Proline-Rich Domain as Transient Folding Chaperone

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

Differences in the free energies between the excited states of Aβ40 and Aβ42 monomers encode their aggregation propensities

(2020) Debayan Chakraborty et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The evolution of the huntingtin-associated protein 40 (HAP40) in conjunction with huntingtin

(2020) Manuel Seefelder et al.   BMC EVOLUTIONARY BIOLOGY

Exploring the binding mechanism between human profilin (PFN1) and polyproline-10 through binding mode screening

(2019) Leili Zhang et al.   JOURNAL OF CHEMICAL PHYSICS

Structural insights into the aggregation mechanism of huntingtin exon 1 protein fragment with different polyQ-lengths

(2019) S. Binny Priya et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

Molecular Dynamics Simulations Applied to Structural and Dynamical Transitions of the Huntingtin Protein: A Review

(2019) Sanda Nastasia Moldovean et al.   ACS Chemical Neuroscience

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

Tadpole-like Conformations of Huntingtin Exon 1 Are Characterized by Conformational Heterogeneity that Persists regardless of Polyglutamine Length

(2018) Estella A. Newcombe et al.   JOURNAL OF MOLECULAR BIOLOGY

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 cryo-electron microscopy structure of huntingtin

(2018) Qiang Guo et al.   NATURE

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

Molecular Mechanism of Stabilizing the Helical Structure of Huntingtin N17 in a Micellar Environment

(2017) Leili Zhang et al.   JOURNAL OF PHYSICAL CHEMISTRY B

Monomeric Huntingtin Exon 1 Has Similar Overall Structural Features for Wild-Type and Pathological Polyglutamine Lengths

(2017) John B. Warner et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Emerging β-Sheet Rich Conformations in Supercompact Huntingtin Exon-1 Mutant Structures

(2017) Hongsuk Kang et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Structure and Dynamics of the Huntingtin Exon-1 N-Terminus: A Solution NMR Perspective

(2017) Maria Baias et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Aggregation landscapes of Huntingtin exon 1 protein fragments and the critical repeat length for the onset of Huntington’s disease

(2017) Mingchen Chen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Fibril polymorphism affects immobilized non-amyloid flanking domains of huntingtin exon1 rather than its polyglutamine core

(2017) Hsiang-Kai Lin et al.   Nature Communications

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients

(2017) Andreas Neueder et al.   Scientific Reports

Huntingtin exon 1 fibrils feature an interdigitated β-hairpin–based polyglutamine core

(2016) Cody L. Hoop et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure of a Single-Chain Fv Bound to the 17 N-Terminal Residues of Huntingtin Provides Insights into Pathogenic Amyloid Formation and Suppression

(2015) Erwin De Genst et al.   JOURNAL OF MOLECULAR BIOLOGY

Too packed to change: side-chain packing and site-specific substitution rates in protein evolution

(2015) María Laura Marcos et al.   PeerJ

Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington’s disease

(2014) Bindu D. Paul et al.   NATURE

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

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

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

β-Hairpin-Mediated Nucleation of Polyglutamine Amyloid Formation

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

Polyproline-II Helix in Proteins: Structure and Function

(2013) Alexei A. Adzhubei et al.   JOURNAL OF MOLECULAR BIOLOGY

Proline: The Distribution, Frequency, Positioning, and Common Functional Roles of Proline and Polyproline Sequences in the Human Proteome

(2013) Alexander A. Morgan et al.   PLoS One

Unmasking the roles of N- and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation

(2013) S. L. Crick et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

Colostral Proline-Rich Polypeptides – Immunoregulatory Properties and Prospects of Therapeutic Use in Alzheimers Disease

(2010) M. Janusz et al.   Current Alzheimer Research

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

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

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

A Collective Variable for the Efficient Exploration of Protein Beta-Sheet Structures: Application to SH3 and GB1

(2009) Fabio Pietrucci et al.   Journal of Chemical Theory and Computation

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

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

Atomistic Simulations of the Effects of Polyglutamine Chain Length and Solvent Quality on Conformational Equilibria and Spontaneous Homodimerization

(2008) Andreas Vitalis et al.   JOURNAL OF MOLECULAR BIOLOGY

Intrabodies Binding the Proline-Rich Domains of Mutant Huntingtin Increase Its Turnover and Reduce Neurotoxicity

(2008) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

Analysis of CCG Repeats in Huntingtin Gene among HD Patients and Normal Populations in Japan

(2007) Saeid Morovvati et al.   ARCHIVES OF MEDICAL RESEARCH

Phylogenetic Comparison of Huntingtin Homologues Reveals the Appearance of a Primitive polyQ in Sea Urchin

(2007) M. Tartari et al.   MOLECULAR BIOLOGY AND EVOLUTION