A survey of protein interactions and posttranslational modifications that influence the polyglutamine diseases

SL Johnson, WL Tsou, MV Prifti, AL Harris… - Frontiers in Molecular …, 2022 - frontiersin.org
The presence and aggregation of misfolded proteins has deleterious effects in the nervous
system. Among the various diseases caused by misfolded proteins is the family of the …

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 …

Polyglutamine disorders: Pathogenesis and potential drug interventions

S Tandon, P Aggarwal, S Sarkar - Life Sciences, 2024 - Elsevier
Polyglutamine/poly (Q) diseases are a group nine hereditary neurodegenerative disorders
caused due to abnormally expanded stretches of CAG trinucleotide in functionally distinct …

Therapeutic approaches to polyglutamine diseases: combating protein misfolding and aggregation

M Herbst, EE Wanker - Current pharmaceutical design, 2006 - ingentaconnect.com
Polyglutamine diseases are autosomal dominant, late-onset neurodegenerative disorders.
Expansion of a polyglutamine (polyQ) tract above a threshold size leads to misfolding and …

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) …

Flanking sequences profoundly alter polyglutamine toxicity in yeast

ML Duennwald, S Jagadish… - Proceedings of the …, 2006 - National Acad Sciences
Protein misfolding is the molecular basis for several human diseases. How the primary
amino acid sequence triggers misfolding and determines the benign or toxic character of the …

Protein misfolding and aggregation as a therapeutic target for polyglutamine diseases

T Takeuchi, Y Nagai - Brain sciences, 2017 - mdpi.com
The polyglutamine (polyQ) diseases, such as Huntington's disease and several types of
spinocerebellar ataxias, are a group of inherited neurodegenerative diseases that are …

Therapeutic opportunities in polyglutamine disease

RE Hughes, JM Olson - Nature medicine, 2001 - nature.com
Polyglutamine diseases comprise a class of familial neurodegenerative disorders caused by
expression of proteins containing expanded polyglutamine tracts. Great progress has been …

Disruption of the toxic conformation of the expanded polyglutamine stretch leads to suppression of aggregate formation and cytotoxicity

HA Popiel, Y Nagai, O Onodera, T Inui… - Biochemical and …, 2004 - Elsevier
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 …

Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity

H Sakahira, P Breuer… - Proceedings of the …, 2002 - National Acad Sciences
The formation of insoluble protein aggregates in neurons is a hallmark of neurodegenerative
diseases caused by proteins with expanded polyglutamine (polyQ) repeats. However, the …