Towards the treatment of polyglutamine diseases: the modulatory role of protein context

AL Robertson, SP Bottomley - Current medicinal chemistry, 2010 - ingentaconnect.com
… that the protein context of the polyQ tract has an essential role in the … role of protein context
in modulating the polyQ diseases and the therapeutic potential of targeting non-polyQ protein

Polyglutamine diseases: emerging concepts in pathogenesis and therapy

J Shao, MI Diamond - Human molecular genetics, 2007 - academic.oup.com
… tract facilitates a crucial conformational transition, which can be influenced by numerous
factors, including the protein context of the repeats and intracellular protein interactions. …

Beyond the Qs in the polyglutamine diseases

HT Orr - Genes & development, 2001 - genesdev.cshlp.org
… of the function of the full-length protein, and (2) a … polyglutamine tract itself regardless of
the protein context in which it is located. In the first case, the altered toxic function of the protein

Pathogenesis of polyglutamine disorders: aggregation revisited

A Michalik, C Van Broeckhoven - Human molecular genetics, 2003 - academic.oup.com
protein context within which it resides. Nevertheless, the protein context does modulate
polyglutamine … For instance, although aggregates of all polyglutamine disease proteins will …

Polyglutamines placed into context

AR La Spada, JP Taylor - Neuron, 2003 - cell.com
… , indicate that host protein context is the key arbiter of polyglutamine disease protein toxicity.
… of nuclear inclusions or the propensity of polyglutamine to aggregate. Indeed, an intimate …

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis) fold

HL Paulson - The American Journal of Human Genetics, 1999 - cell.com
disease process; next, it will be necessary to explain how specific changes in disease
proteins … they override their specific disease context, causing a more widespread “glutaminopathy.” …

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
… and this work was on a model protein and not on a polyglutamine disease causing protein.
We used time-… have similar toxic structures despite the context of their amino acids sequence. …

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

PO Bauer, N Nukina - Journal of neurochemistry, 2009 - Wiley Online Library
… the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of
these pathogenic proteins leads to PolyQ diseases, such as Huntington’s disease or several …

Polyglutamine expansion diseases: More than simple repeats

A Silva, AV de Almeida, S Macedo-Ribeiro - Journal of Structural Biology, 2018 - Elsevier
context of the full-length proteins, as implied by variations in the length of the polyQ tract
required to trigger the onset of a given polyQ disease. … Polyglutamine diseases are a large group …

Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease

JF Gusella, ME MacDonald - Nature Reviews Neuroscience, 2000 - nature.com
… depends on protein context. These curves imply that in this class of inherited neurodegenerative
… on the protein in which it is contained. Most patients with polyglutamine diseases are …