Polyglutamine aggregation in Huntington and related diseases

S Polling, AF Hill, DM Hatters - Tandem Repeat Polymorphisms: Genetic …, 2012 - Springer
Polyglutamine (polyQ)-expansions in differentproteins cause nine neurodegenerative
diseases. While polyQ aggregation is a key pathological hallmark of these diseases, how …

Polyglutamine protein trafficking and neurodegeneration

JR McGuire, SH Li, XJ Li - Current Genomics, 2005 - ingentaconnect.com
Polyglutamine (polyQ) expansion causes nine inherited neurodegenerative disorders,
including Huntington's Disease, Spinobulbar Muscular Atrophy, Dentatorubral …

Multi-domain misfolding: understanding the aggregation pathway of polyglutamine proteins

HM Saunders, SP Bottomley - Protein engineering, design & …, 2009 - academic.oup.com
The polyglutamine (polyQ) diseases consist of nine neurodegenerative diseases in which a
polyQ tract expansion leads to protein misfolding and subsequent aggregation. Even when …

Polyglutamine expansion diseases: More than simple repeats

A Silva, AV de Almeida, S Macedo-Ribeiro - Journal of Structural Biology, 2018 - Elsevier
Polyglutamine (polyQ) repeat-containing proteins are widespread in the human proteome
but only nine of them are associated with highly incapacitating neurodegenerative disorders …

Molecular pathways to polyglutamine aggregation

AL Robertson, SP Bottomley - Tandem Repeat Polymorphisms: Genetic …, 2012 - Springer
Over 100 human cellular proteins contain a repetitive polyglutamine tract, however, only
nine of these proteins are associated with disease. In these proteins, the expanded polyQ …

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) …

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 …

Proteomics of polyglutamine aggregates

K Mitsui, H Doi, N Nukina - Methods in enzymology, 2006 - Elsevier
In nine members of polyglutamine (polyQ) diseases, CAG repeat expansions of their
responsible genes are observed. The disease is considered to be caused by the formation …

A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo

X Zhang, DL Smith, AB Meriin… - Proceedings of the …, 2005 - National Acad Sciences
Polyglutamine (polyQ) disorders, including Huntington's disease (HD), are caused by
expansion of polyQ-encoding repeats within otherwise unrelated gene products. In polyQ …

[HTML][HTML] 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 …