Pathogenic and non-pathogenic polyglutamine tracts have similar structural properties: towards a length-dependent toxicity gradient
FAC Klein, A Pastore, L Masino, G Zeder-Lutz… - Journal of molecular …, 2007 - Elsevier
Abnormally expanded polyglutamine (polyQ) tracts provide a gain of toxic functions to nine
otherwise unrelated human proteins and induce progressive neurodegenerative diseases …
otherwise unrelated human proteins and induce progressive neurodegenerative diseases …
A network of protein interactions determines polyglutamine toxicity
ML Duennwald, S Jagadish… - Proceedings of the …, 2006 - National Acad Sciences
Several neurodegenerative diseases are associated with the toxicity of misfolded proteins.
This toxicity must arise from a combination of the amino acid sequences of the misfolded …
This toxicity must arise from a combination of the amino acid sequences of the misfolded …
Identifying polyglutamine protein species in situ that best predict neurodegeneration
J Miller, M Arrasate, E Brooks, CP Libeu… - Nature chemical …, 2011 - nature.com
Polyglutamine (polyQ) stretches exceeding a threshold length confer a toxic function to
proteins that contain them and cause at least nine neurological disorders. The basis for this …
proteins that contain them and cause at least nine neurological disorders. The basis for this …
Towards the treatment of polyglutamine diseases: the modulatory role of protein context
AL Robertson, SP Bottomley - Current medicinal chemistry, 2010 - ingentaconnect.com
Protein aggregation is a key mechanism involved in neurodegeneration associated with
Alzheimer's, Parkinson's and Huntington's diseases. Nine diseases (including Huntington's) …
Alzheimer's, Parkinson's and Huntington's diseases. Nine diseases (including Huntington's) …
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
T Takahashi, S Kikuchi, S Katada… - Human molecular …, 2008 - academic.oup.com
Expanded polyglutamine (polyQ) repeats cause neurodegenerative disorders, but their
cytotoxic structures remain to be elucidated. Although soluble polyQ oligomers have been …
cytotoxic structures remain to be elucidated. Although soluble polyQ oligomers have been …
The rate of polyQ-mediated aggregation is dramatically affected by the number and location of surrounding domains
AL Robertson, MA Bate, AM Buckle… - Journal of molecular …, 2011 - Elsevier
The nine polyglutamine (polyQ) neurodegenerative diseases are caused in part by a gain-of-
function mechanism involving protein misfolding, the deposition of β-sheet-rich aggregates …
function mechanism involving protein misfolding, the deposition of β-sheet-rich aggregates …
Neurotoxic protein oligomerisation associated with polyglutamine diseases
SL Hands, A Wyttenbach - Acta neuropathologica, 2010 - Springer
Polyglutamine (polyQ) diseases are associated with a CAG/polyQ expansion mutation in
unrelated proteins. Upon elongation of the glutamine tract, disease proteins aggregate …
unrelated proteins. Upon elongation of the glutamine tract, disease proteins aggregate …
Disruption of the toxic conformation of the expanded polyglutamine stretch leads to suppression of aggregate formation and cytotoxicity
The polyglutamine (polyQ) diseases are a class of inherited neurodegenerative diseases
including Huntington's disease, caused by the expansion of a polyQ stretch within each …
including Huntington's disease, caused by the expansion of a polyQ stretch within each …
Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence
R Wetzel - Journal of molecular biology, 2012 - Elsevier
Polyglutamine (polyQ) sequences of unknown normal function are present in a significant
number of proteins, and their repeat expansion is associated with a number of genetic …
number of proteins, and their repeat expansion is associated with a number of genetic …
A toxic monomeric conformer of the polyglutamine protein
Polyglutamine (polyQ) diseases are classified as conformational neurodegenerative
diseases, like Alzheimer and Parkinson diseases, and they are caused by proteins with an …
diseases, like Alzheimer and Parkinson diseases, and they are caused by proteins with an …