Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: Implication in a loss-of-function pathology

Structural motif of polyglutamine amyloid fibrils discerned with mixed-isotope infrared spectroscopy

(2014) L. E. Buchanan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structural and mechanistic insights into the arginine/lysine-rich peptide motifs that interact with P97/VCP

(2013) Shuai Liu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Length-dependent conformational transitions of polyglutamine repeats as molecular origin of fibril initiation

(2013) Benjamin S. Heck et al.   BIOPHYSICAL CHEMISTRY

PolyQ Proteins Interfere with Nuclear Degradation of Cytosolic Proteins by Sequestering the Sis1p Chaperone

(2013) Sae-Hun Park et al.   CELL

NEDD8 Ultimate Buster-1 Long (NUB1L) Protein Promotes Transfer of NEDD8 to Proteasome for Degradation through the P97UFD1/NPL4Complex

(2013) Shuai Liu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

(2013) Kyota Fujita et al.   Nature Communications

Length of polyglutamine tract affects secondary and tertiary structures of huntingtin protein

(2012) Shivang N. Vachharajani et al.   INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES

Cell biology of spinocerebellar ataxia

(2012) Harry T. Orr   JOURNAL OF CELL BIOLOGY

PolyQ disease: misfiring of a developmental cell death program?

(2012) Elyse S. Blum et al.   TRENDS IN CELL BIOLOGY

Length of the active-site crossover loop defines the substrate specificity of ubiquitin C-terminal hydrolases for ubiquitin chains

(2011) Zi-Ren Zhou et al.   BIOCHEMICAL JOURNAL

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

(2011) Heidi Olzscha et al.   CELL

Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions

(2011) Aurélien Davranche et al.   HUMAN MOLECULAR GENETICS

Interaction with Polyglutamine-expanded Huntingtin Alters Cellular Distribution and RNA Processing of Huntingtin Yeast Two-hybrid Protein A (HYPA)

(2011) Ya-Jun Jiang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks

(2011) Klara Acs et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Domain Analysis Reveals That a Deubiquitinating Enzyme USP13 Performs Non-Activating Catalysis for Lys63-Linked Polyubiquitin

(2011) Yu-Hang Zhang et al.   PLoS One

Axonal inclusions in spinocerebellar ataxia type 3

(2010) Kay Seidel et al.   ACTA NEUROPATHOLOGICA

Functional interactions as a survival strategy against abnormal aggregation

(2010) Masino Laura et al.   FASEB JOURNAL

Nuclear Aggregation of Polyglutamine-expanded Ataxin-3

(2010) Peter Breuer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Understanding the Role of the Josephin Domain in the PolyUb Binding and Cleavage Properties of Ataxin-3

(2010) Giuseppe Nicastro et al.   PLoS One

Structural Transformation of the Tandem Ubiquitin-Interacting Motifs in Ataxin-3 and Their Cooperative Interactions with Ubiquitin Chains

(2010) Ai-Xin Song et al.   PLoS One

Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties

(2010) Ginny Marie Harris et al.   PLoS One

Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sites

(2009) Giuseppe Nicastro et al.   BIOPOLYMERS

Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3

(2009) Joonil Jung et al.   HUMAN MOLECULAR GENETICS

CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3

(2009) Thorsten Mueller et al.   HUMAN MOLECULAR GENETICS