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

Miles to go (mtgo) encodes FNDC3 proteins that interact with the chaperonin subunit CCT3 and are required for NMJ branching and growth in Drosophila

(2018) Adeela Syed et al.   DEVELOPMENTAL BIOLOGY

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

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

CCT complex restricts neuropathogenic protein aggregation via autophagy

(2016) Mariana Pavel et al.   Nature Communications

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

(2016) Ravi Vijayvargia 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

Compartment-specific aggregases direct distinct nuclear and cytoplasmic aggregate deposition

(2015) S. B. Miller et al.   EMBO JOURNAL

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

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

Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Spanin Vivo

(2015) Robert O'Brien et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Sorting out the trash: the spatial nature of eukaryotic protein quality control

(2014) Emily M Sontag et al.   CURRENT OPINION IN CELL BIOLOGY

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

Potential function for the Huntingtin protein as a scaffold for selective autophagy

(2014) Joseph Ochaba et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Preventing Formation of Toxic N-Terminal Huntingtin Fragments Through Antisense Oligonucleotide-Mediated Protein Modification

(2014) Melvin M. Evers et al.   Nucleic Acid Therapeutics

Secretory competence in a gateway endocrine cell conferred by the nuclear receptor βFTZ-F1 enables stage-specific ecdysone responses throughout development in Drosophila

(2013) Kook-Ho Cho et al.   DEVELOPMENTAL BIOLOGY

Live cell imaging and biophotonic methods reveal two types of mutant huntingtin inclusions

(2013) Nicholas S. Caron et al.   HUMAN MOLECULAR GENETICS

The DNAJB6 and DNAJB8 Protein Chaperones Prevent Intracellular Aggregation of Polyglutamine Peptides

(2013) Judith Gillis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A flexible polyglutamine hinge opens new doors for understanding huntingtin function: Fig. 1.

(2013) Flaviano Giorgini   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin

(2013) Nicholas Stephane Caron et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Aberrantly splicedHTT,a new player in Huntington’s disease pathogenesis

(2013) Theresa A Gipson et al.   RNA Biology

Protein aggregates in Huntington's disease

(2011) Montserrat Arrasate et al.   EXPERIMENTAL NEUROLOGY

Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment

(2011) Andrew T.N. Tebbenkamp et al.   HUMAN MOLECULAR GENETICS

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

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

A Systematic Screen for Tube Morphogenesis and Branching Genes in the Drosophila Tracheal System

(2011) Amin S. Ghabrial et al.   PLoS Genetics

Does Huntingtin play a role in selective macroautophagy?

(2010) Joan S. Steffan   CELL CYCLE

Polyglutamine toxicity in non-neuronal cells

(2010) Jennifer W Bradford et al.   CELL RESEARCH

Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

(2010) Christian Landles et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

Modulation of Mutant Huntingtin N-Terminal Cleavage and Its Effect on Aggregation and Cell Death

(2010) Katrin Juenemann et al.   NEUROTOXICITY RESEARCH

Interaction between Drosophila bZIP proteins Atf3 and Jun prevents replacement of epithelial cells during metamorphosis

(2009) P. Sekyrova et al.   DEVELOPMENT

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

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

Mimicking proteasomal release of polyglutamine peptides initiates aggregation and toxicity

(2009) M. Raspe et al.   JOURNAL OF CELL SCIENCE

Beyond the brain: widespread pathology in Huntington's disease

(2009) Jorien MM van der Burg et al.   LANCET NEUROLOGY

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

Detection of Alpha-Rod Protein Repeats Using a Neural Network and Application to Huntingtin

(2009) Gareth A. Palidwor et al.   PLoS Computational Biology

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