Chaperones in polyglutamine aggregation: beyond the Q-stretch

EFE Kuiper, EP De Mattos, LB Jardim… - Frontiers in …, 2017 - frontiersin.org
Expanded polyglutamine (polyQ) stretches in at least nine unrelated proteins lead to
inherited neuronal dysfunction and degeneration. The expansion size in all diseases …

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 …

Canadian Association of Neurosciences Review: polyglutamine expansion neurodegenerative diseases

R Truant, LA Raymond, J Xia, D Pinchev… - Canadian journal of …, 2006 - cambridge.org
Since the early 1990s, DNA triplet repeat expansions have been found to be the cause in an
ever increasing number of genetic neurologic diseases. A subset of this large family of …

Polyglutamine diseases: where does toxicity come from? what is toxicity? where are we going?

T Takahashi, S Katada… - Journal of molecular cell …, 2010 - academic.oup.com
Although the genetic basis of polyglutamine diseases has been recognized for 20 years,
their molecular basis is still unclear. We have no therapeutic strategies for these intractable …

Polyglutamine disorders including huntington's disease

SV Todi, AJ Williams, HL Paulson - Molecular neurology, 2007 - books.google.com
At least nine inherited neurodegenerative disorders are caused by expansion of a CAG
repeat in the protein-coding region of the respective disease genes (see Table 17.1) …

Polyglutamine toxicity in non-neuronal cells

JW Bradford, S Li, XJ Li - Cell research, 2010 - nature.com
The neurodegenerative polyglutamine diseases are caused by an expansion of unstable
polyglutamine repeats in various disease proteins. Although these mutant proteins are …

[HTML][HTML] Pharmacological protein targets in polyglutamine diseases: mutant polypeptides and their interactors

BA Margulis, V Vigont, VF Lazarev, EV Kaznacheyeva… - FEBS letters, 2013 - Elsevier
Polyglutamine diseases are a group of pathologies affecting different parts of the brain and
causing dysfunction and atrophy of certain neural cell populations. These diseases stem …

The expanding clinical universe of polyglutamine disease

S Huang, S Zhu, XJ Li, S Li - The Neuroscientist, 2019 - journals.sagepub.com
Polyglutamine (polyQ) diseases are a group of hereditary neurodegenerative disorders
caused by expansion of unstable polyQ repeats in their associated disease proteins. To …

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 …

Conformation polymorphism of polyglutamine proteins

X Feng, S Luo, B Lu - Trends in biochemical sciences, 2018 - cell.com
Expanded polyglutamine (polyQ) stretches within endogenous proteins cause at least nine
human diseases. The structural basis of polyQ pathogenesis is the key to understanding …