[HTML][HTML] The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils

D Bulone, L Masino, DJ Thomas, PL San Biagio… - PloS one, 2006 - journals.plos.org
Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders caused by the
expansion of CAG codon repeats, which code for polyQ in the corresponding gene products …

Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model

HR Brignull, FE Moore, SJ Tang… - Journal of …, 2006 - Soc Neuroscience
The basis of neuron-specific pathogenesis, resulting from the expression of misfolded
proteins, is poorly understood and of central importance to an understanding of the cell-type …

Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease

JF Gusella, ME MacDonald - Nature Reviews Neuroscience, 2000 - nature.com
Two decades ago, molecular genetic analysis provided a new approach for defining the
roots of inherited disorders. This strategy has proved particularly powerful because, with …

[HTML][HTML] Polyglutamines placed into context

AR La Spada, JP Taylor - Neuron, 2003 - cell.com
Nine inherited neurodegenerative disorders result from polyglutamine expansions. Two
recently published papers on spinocerebellar ataxia type 1, together with studies on …

Amino acid sequence requirements of peptides that inhibit polyglutamine-protein aggregation and cell death

H Ren, Y Nagai, T Tucker, WJ Strittmatter… - Biochemical and …, 2001 - Elsevier
Proteins with expanded polyglutamine domains cause eight inherited neurodegenerative
diseases including Huntington's disease. In a previous paper, we identified peptides that …

Assessing a peptidylic inhibitor-based therapeutic approach that simultaneously suppresses polyglutamine RNA-and protein-mediated toxicities in patient cells and …

Q Zhang, H Tsoi, S Peng, PP Li… - Disease models & …, 2016 - journals.biologists.com
Polyglutamine (polyQ) diseases represent a group of progressive neurodegenerative
disorders that are caused by abnormal expansion of CAG triplet nucleotides in disease …

[HTML][HTML] Oxidative stress and neurodegeneration: Interconnected processes in polyq diseases

I Gkekas, A Gioran, MK Boziki, N Grigoriadis… - Antioxidants, 2021 - mdpi.com
Neurodegenerative polyglutamine (polyQ) disorders are caused by trinucleotide repeat
expansions within the coding region of disease-causing genes. PolyQ-expanded proteins …

The CAG/polyglutamine tract diseases: gene products and molecular pathogenesis

BT Koshy, HY Zoghbi - Brain Pathology, 1997 - Wiley Online Library
In the past few years, a new type of genetic mutation, expansion of trinucleotide repeats, has
been shown to cause neurologic disease. This new class of mutations was first identified in …

[HTML][HTML] Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties

GM Harris, K Dodelzon, L Gong, P Gonzalez-Alegre… - PloS one, 2010 - journals.plos.org
Protein context clearly influences neurotoxicity in polyglutamine diseases, but the
contribution of alternative splicing to this phenomenon has rarely been investigated. Ataxin …

Modifiers and mechanisms of multi-system polyglutamine neurodegenerative disorders: lessons from fly models

M Mallik, SC Lakhotia - Journal of genetics, 2010 - Springer
Polyglutamine (polyQ) diseases, resulting from a dynamic expansion of glutamine repeats in
a polypeptide, are a class of genetically inherited late onset neurodegenerative disorders …