Aggregation formation in the polyglutamine diseases: Protection at a cost?

Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy

(2013) Bilal Malik et al.   BRAIN

β-Hairpin-Mediated Nucleation of Polyglutamine Amyloid Formation

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

Study of the aggregation mechanism of polyglutamine peptides using replica exchange molecular dynamics simulations

(2013) Miki Nakano et al.   JOURNAL OF MOLECULAR MODELING

p62/SQSTM1 Differentially Removes the Toxic Mutant Androgen Receptor via Autophagy and Inclusion Formation in a Spinal and Bulbar Muscular Atrophy Mouse Model

(2013) H. Doi et al.   JOURNAL OF NEUROSCIENCE

Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration

(2013) Naohide Kondo et al.   Nature Communications

Pharmacological Tuning of Heat Shock Protein 70 Modulates Polyglutamine Toxicity and Aggregation

(2012) Sidhartha M. Chafekar et al.   ACS Chemical Biology

Neuronal aggregates are associated with phenotypic onset in the R6/2 Huntington's disease transgenic mouse

(2012) Randi-Michelle Cowin et al.   BEHAVIOURAL BRAIN RESEARCH

Toxic and non-toxic aggregates from the SBMA and normal forms of androgen receptor have distinct oligomeric structures

(2012) Tobias Jochum et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Ubiquitination is involved in secondary growth, not initial formation of polyglutamine protein aggregates in C. elegans

(2012) Gregory A Skibinski et al.   BMC CELL BIOLOGY

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

Physical Chemistry of Polyglutamine: Intriguing Tales of a Monotonous Sequence

(2012) Ronald Wetzel   JOURNAL OF MOLECULAR BIOLOGY

Methylene Blue Modulates Huntingtin Aggregation Intermediates and Is Protective in Huntington's Disease Models

(2012) E. M. Sontag et al.   JOURNAL OF NEUROSCIENCE

Protein aggregates in Huntington's disease

(2011) Montserrat Arrasate et al.   EXPERIMENTAL NEUROLOGY

Identifying polyglutamine protein species in situ that best predict neurodegeneration

(2011) Jason Miller et al.   Nature Chemical Biology

Macroautophagy Is Regulated by the UPR–Mediator CHOP and Accentuates the Phenotype of SBMA Mice

(2011) Zhigang Yu et al.   PLoS Genetics

Neurotoxic protein oligomerisation associated with polyglutamine diseases

(2010) Sarah L. Hands et al.   ACTA NEUROPATHOLOGICA

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

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

Inhibition of Hsp70 by Methylene Blue Affects Signaling Protein Function and Ubiquitination and Modulates Polyglutamine Protein Degradation

(2010) Adrienne M. Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp70 and Hsp40 Functionally Interact with Soluble Mutant Huntingtin Oligomers in a Classic ATP-dependent Reaction Cycle

(2010) Gregor P. Lotz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy

(2010) Isabella Palazzolo et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Assessing mutant huntingtin fragment and polyglutamine aggregation by atomic force microscopy

(2010) Kathleen A. Burke et al.   METHODS

Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein

(2010) Peter O Bauer et al.   NATURE BIOTECHNOLOGY

17-AAG increases autophagic removal of mutant androgen receptor in spinal and bulbar muscular atrophy

(2010) Paola Rusmini et al.   NEUROBIOLOGY OF DISEASE

Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?

(2010) T. Takahashi et al.   Journal of Molecular Cell Biology

Autophagy and access:  Understanding the role of androgen receptor subcellular localization in SBMA

(2009) Heather Montie et al.   Autophagy

Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy

(2009) Heather L. Montie 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

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

(2009) Justin Legleiter et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

(2009) Peter O. Bauer et al.   JOURNAL OF NEUROCHEMISTRY

Loss of Hsp70 Exacerbates Pathogenesis But Not Levels of Fibrillar Aggregates in a Mouse Model of Huntington's Disease

(2009) J. L. Wacker et al.   JOURNAL OF NEUROSCIENCE

Conformational Targeting of Fibrillar Polyglutamine Proteins in Live Cells Escalates Aggregation and Cytotoxicity

(2009) Erik Kvam et al.   PLoS One

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

HSP70 interacting protein prevents the accumulation of inclusions in polyglutamine disease

(2008) Joanna L. Howarth et al.   JOURNAL OF NEUROCHEMISTRY

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis

(2008) Aislinn J. Williams et al.   NEUROBIOLOGY OF DISEASE

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

Suppression of Neurodegeneration and Increased Neurotransmission Caused by Expanded Full-Length Huntingtin Accumulating in the Cytoplasm

(2008) Eliana Romero et al.   NEURON

Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington's disease reveals time-dependent aggregate formation that transiently delays cell death

(2007) B. Gong et al.   BRAIN RESEARCH BULLETIN