Proteins containing expanded polyglutamine tracts and neurodegenerative disease
A Adegbuyiro, F Sedighi, AW Pilkington IV… - Biochemistry, 2017 - ACS Publications
Several hereditary neurological and neuromuscular diseases are caused by an abnormal
expansion of trinucleotide repeats. To date, there have been 10 of these trinucleotide repeat …
expansion of trinucleotide repeats. To date, there have been 10 of these trinucleotide repeat …
SCA3: neurological features, pathogenesis and animal models
O Riess, U Rüb, A Pastore, P Bauer, L Schöls - The Cerebellum, 2008 - Springer
The most frequent subtype of autosomal dominant inherited spinocerebellar ataxias is
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …
Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation
S Chen, FA Ferrone, R Wetzel - Proceedings of the …, 2002 - National Acad Sciences
In Huntington's Disease and related expanded CAG repeat diseases, a polyglutamine [poly
(Gln)] sequence containing 36 repeats in the corresponding disease protein is benign …
(Gln)] sequence containing 36 repeats in the corresponding disease protein is benign …
ABSINTH: a new continuum solvation model for simulations of polypeptides in aqueous solutions
A new implicit solvation model for use in Monte Carlo simulations of polypeptides is
introduced. The model is termed ABSINTH for self‐Assembly of Biomolecules Studied by an …
introduced. The model is termed ABSINTH for self‐Assembly of Biomolecules Studied by an …
Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells
W Yang, JR Dunlap, RB Andrews… - Human molecular …, 2002 - academic.oup.com
A number of observations point to the aggregation of expanded polyglutamine [poly (Q)]-
containing proteins as playing a central role in the etiology of Huntington's disease (HD) and …
containing proteins as playing a central role in the etiology of Huntington's disease (HD) and …
Secondary structure of Huntingtin amino-terminal region
MW Kim, Y Chelliah, SW Kim, Z Otwinowski… - Structure, 2009 - cell.com
Huntington's disease is a genetic neurodegenerative disorder resulting from polyglutamine
(polyQ) expansion (> 36Q) within the first exon of Huntingtin (Htt) protein. We applied X-ray …
(polyQ) expansion (> 36Q) within the first exon of Huntingtin (Htt) protein. We applied X-ray …
Identifying polyglutamine protein species in situ that best predict neurodegeneration
J Miller, M Arrasate, E Brooks, CP Libeu… - Nature chemical …, 2011 - nature.com
Polyglutamine (polyQ) stretches exceeding a threshold length confer a toxic function to
proteins that contain them and cause at least nine neurological disorders. The basis for this …
proteins that contain them and cause at least nine neurological disorders. The basis for this …
Fluorescence correlation spectroscopy shows that monomeric polyglutamine molecules form collapsed structures in aqueous solutions
We have used fluorescence correlation spectroscopy measurements to quantify the
hydrodynamic sizes of monomeric polyglutamine as a function of chain length (N) by …
hydrodynamic sizes of monomeric polyglutamine as a function of chain length (N) by …
Oligoproline effects on polyglutamine conformation and aggregation
A Bhattacharyya, AK Thakur, VM Chellgren… - Journal of molecular …, 2006 - Elsevier
There are nine known expanded CAG repeat neurological diseases, including Huntington's
disease (HD), each involving the repeat expansion of polyglutamine (polyGln) in a different …
disease (HD), each involving the repeat expansion of polyglutamine (polyGln) in a different …
Mutant huntingtin is cleared from the brain via active mechanisms in Huntington disease
Huntington disease (HD) is a neurodegenerative disease caused by a CAG trinucleotide
repeat expansion in the huntingtin (HTT) gene. Therapeutics that lower HTT have shown …
repeat expansion in the huntingtin (HTT) gene. Therapeutics that lower HTT have shown …