4.6 Review

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

Related references

Note: Only part of the references are listed.
Article Clinical Neurology

Phase 2 Trial of Leuprorelin in Patients with Spinal and Bulbar Muscular Atrophy

Haruhiko Banno et al.

ANNALS OF NEUROLOGY (2009)

Review Biochemistry & Molecular Biology

Pathogenic Mechanisms of a Polyglutamine-mediated Neurodegenerative Disease, Spinocerebellar Ataxia Type 1

Huda Y. Zoghbi et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2009)

Review Multidisciplinary Sciences

The promises and pitfalls of RNA-interference-based therapeutics

Daniela Castanotto et al.

NATURE (2009)

Article Biotechnology & Applied Microbiology

Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs

Jiaxin Hu et al.

NATURE BIOTECHNOLOGY (2009)

Article Multidisciplinary Sciences

Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity

Srinivasa Subramaniam et al.

SCIENCE (2009)

Article Biochemistry & Molecular Biology

Polyglutamine expansion reduces the association of TATA-binding protein with DNA and induces DNA binding-independent neurotoxicity

Meyer J. Friedman et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2008)

Article Biochemistry & Molecular Biology

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

Toshiaki Takahashi et al.

HUMAN MOLECULAR GENETICS (2008)

Article Neurosciences

A Mutant ataxin-3 fragment N-terminal to amino acid 190 disease-like transgenic mice

Veronica F. Colomer Gould et al.

NEUROBIOLOGY OF DISEASE (2007)

Article Multidisciplinary Sciences

Transvascular delivery of small interfering RNA to the central nervous system

Priti Kumar et al.

NATURE (2007)

Article Biochemistry & Molecular Biology

Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity

Isabella Palazzolo et al.

HUMAN MOLECULAR GENETICS (2007)

Article Multidisciplinary Sciences

Huntingtin facilitates dynein/dynactin-mediated vesicle transport

Juliane P. Caviston et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2007)

Article Multidisciplinary Sciences

OGG1 initiates age-dependent CAG trinucleotide expansion in somatic cells

Irina V. Kovtun et al.

NATURE (2007)

Article Biochemistry & Molecular Biology

A toxic monomeric conformer of the polyglutamine protein

Yoshitaka Nagai et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2007)

Article Biochemistry & Molecular Biology

Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy

Mei Li et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2007)

Article Neurosciences

Ubiquitin-conjugating enzyme E2-25K increases aggregate formation and cell death in polyglutamine diseases

Remko de Pril et al.

MOLECULAR AND CELLULAR NEUROSCIENCE (2007)

Review Multidisciplinary Sciences

A century-old debate on protein aggregation and neurodegeneration enters the clinic

Peter T. Lansbury et al.

NATURE (2006)

Review Multidisciplinary Sciences

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

Michael T. Lin et al.

NATURE (2006)

Article Biochemistry & Molecular Biology

Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers

Christian Behrends et al.

MOLECULAR CELL (2006)

Article Biochemistry & Molecular Biology

CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation

Ismael Al-Ramahi et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2006)

Article Biochemistry & Molecular Biology

Biologically active molecules that reduce polyglutamine aggregation and toxicity

Urvee A. Desai et al.

HUMAN MOLECULAR GENETICS (2006)

Article Neurosciences

Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function

Anat Yanai et al.

NATURE NEUROSCIENCE (2006)

Article Biochemistry & Molecular Biology

Interaction of huntingtin fragments with brain membranes -: clues to early dysfunction in Huntington's disease

J Suopanki et al.

JOURNAL OF NEUROCHEMISTRY (2006)

Article Multidisciplinary Sciences

Evidence that Perutz's double-β-stranded subunit structure for β-amyloids also applies to their channel-forming structures in membranes

SJ Singer et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)

Article Neurosciences

CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo

VM Miller et al.

JOURNAL OF NEUROSCIENCE (2005)

Article Biochemistry & Molecular Biology

17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration

M Waza et al.

NATURE MEDICINE (2005)

Review Genetics & Heredity

Diseases of unstable repeat expansion: Mechanisms and common principles

JR Gatchel et al.

NATURE REVIEWS GENETICS (2005)

Article Multidisciplinary Sciences

Suppression of Huntington's disease pathology in Drosophila by human single-chain Fv antibodies

WJ Wolfgang et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)

Article Multidisciplinary Sciences

Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration

VB Palhan et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)

Article Biochemistry & Molecular Biology

Calcium dysregulation and membrane disruption as a ubiquitous neurotoxic mechanism of soluble amyloid oligomers

A Demuro et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2005)

Article Multidisciplinary Sciences

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

SQ Harper et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)

Article Biochemistry & Molecular Biology

Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes

NR Jana et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2005)

Article Multidisciplinary Sciences

Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease

TS Tang et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)

Article Biochemistry & Molecular Biology

Polyglutamine expansion diseases: failing to deliver

G Morfini et al.

TRENDS IN MOLECULAR MEDICINE (2005)

Review Neurosciences

Modulation of neurodegeneration by molecular chaperones

PJ Muchowski et al.

NATURE REVIEWS NEUROSCIENCE (2005)

Article Biochemistry & Molecular Biology

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

M Tanaka et al.

NATURE MEDICINE (2004)

Article Biochemistry & Molecular Biology

RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia

HB Xia et al.

NATURE MEDICINE (2004)

Review Biochemistry & Molecular Biology

Protein aggregation and neurodegenerative disease

CA Ross et al.

NATURE MEDICINE (2004)

Article Biochemistry & Molecular Biology

Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3

SJS Berke et al.

JOURNAL OF NEUROCHEMISTRY (2004)

Article Biochemistry & Molecular Biology

CHIP activates HSF1 and confers protection against apoptosis and cellular stress

Q Dai et al.

EMBO JOURNAL (2003)

Review Cell Biology

Regulation of cell death: The calcium-apoptosis link

S Orrenius et al.

NATURE REVIEWS MOLECULAR CELL BIOLOGY (2003)

Article Biochemistry & Molecular Biology

Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila

Y Nagai et al.

HUMAN MOLECULAR GENETICS (2003)

Article Multidisciplinary Sciences

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

E Hockly et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)

Article Multidisciplinary Sciences

Mutational analysis of the structural organization of polyglutamine aggregates

AK Thakur et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)

Review Biotechnology & Applied Microbiology

DNA repair in neural cells: basic science and clinical implications

PJ Brooks

MUTATION RESEARCH-FUNDAMENTAL AND MOLECULAR MECHANISMS OF MUTAGENESIS (2002)

Article Biochemistry & Molecular Biology

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

MA Poirier et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2002)

Article Multidisciplinary Sciences

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic model of Huntington's disease

CD Keene et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)

Article Multidisciplinary Sciences

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

AW Dunah et al.

SCIENCE (2002)

Article Multidisciplinary Sciences

Partitioning of lipid-modified monomeric GFPs into membrane microdomains of live cells

DA Zacharias et al.

SCIENCE (2002)

Article Multidisciplinary Sciences

Amyloid fibers are water-filled nanotubes

MF Perutz et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2002)

Article Biochemistry & Molecular Biology

Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice

ZX Yu et al.

HUMAN MOLECULAR GENETICS (2002)

Review Cell Biology

Live-cell fluorescent biosensors for activated signaling proteins

K Hahn et al.

CURRENT OPINION IN CELL BIOLOGY (2002)

Review Biochemistry & Molecular Biology

Role and fate of PML nuclear bodies in response to interferon and viral infections

T Regad et al.

ONCOGENE (2001)

Article Multidisciplinary Sciences

An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates parallel β-fibrils

AE Bevivino et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)

Article Multidisciplinary Sciences

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

C Zuccato et al.

SCIENCE (2001)

Article Multidisciplinary Sciences

Impairment of the ubiquitin-proteasome system by protein aggregation

NF Bence et al.

SCIENCE (2001)

Review Cell Biology

Beyond the Qs in the polyglutamine diseases

HT Orr

GENES & DEVELOPMENT (2001)

Article Clinical Neurology

Pathology of CAG repeat diseases

M Yamada et al.

NEUROPATHOLOGY (2000)

Article Multidisciplinary Sciences

Genetic suppression of polyglutamine toxicity in Drosophila

P Kazemi-Esfarjani et al.

SCIENCE (2000)