Results 31 to 40 of about 11,293 (160)

Nuclear accumulation of polyglutamine disease proteins and neuropathology

Molecular Brain, 2009
There are nine inherited neurodegenerative disorders caused by polyglutamine (polyQ) expansion in various disease proteins. Although these polyglutamine proteins have different functions and are localized in different subcellular regions, all the polyQ ...
Havel Lauren S, Li Shihua, Li Xiao-Jiang
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Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. [PDF]

PLoS ONE, 2010
Protein context clearly influences neurotoxicity in polyglutamine diseases, but the contribution of alternative splicing to this phenomenon has rarely been investigated.
Ginny Marie Harris, Katerina Dodelzon, Lijie Gong, Pedro Gonzalez-Alegre, Henry L Paulson   +4 more
doaj   +1 more source

Activation of p38MAPK contributes to expanded polyglutamine-induced cytotoxicity.

PLoS ONE, 2008
BackgroundThe signaling pathways that may modulate the pathogenesis of diseases induced by expanded polyglutamine proteins are not well understood.Methodologies/principal findingsHerein we demonstrate that expanded polyglutamine protein cytotoxicity is ...
Maria Tsirigotis, R Mitchell Baldwin, Matthew Y Tang, Ian A J Lorimer, Douglas A Gray   +4 more
doaj   +1 more source

Conformational targeting of fibrillar polyglutamine proteins in live cells escalates aggregation and cytotoxicity.

PLoS ONE, 2009
BackgroundMisfolding- and aggregation-prone proteins underlying Parkinson's, Huntington's and Machado-Joseph diseases, namely alpha-synuclein, huntingtin, and ataxin-3 respectively, adopt numerous intracellular conformations during pathogenesis ...
Erik Kvam, Brent L Nannenga, Min S Wang, Zongjian Jia, Michael R Sierks, Anne Messer   +5 more
doaj   +1 more source

RACK1 modulates polyglutamine-induced neurodegeneration by promoting ERK degradation in Drosophila.

PLoS Genetics, 2021
Polyglutamine diseases are neurodegenerative diseases caused by the expansion of polyglutamine (polyQ) tracts within different proteins. Although multiple pathways have been found to modulate aggregation of the expanded polyQ proteins, the mechanisms by ...
Jun Xie, Yongchao Han, Tao Wang
doaj   +1 more source

Selective suppression of polyglutamine-expanded protein by lipid nanoparticle-delivered siRNA targeting CAG expansions in the mouse CNS

Molecular Therapy: Nucleic Acids, 2021
Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders caused by expansion of cytosine-adenine-guanine (CAG)-trinucleotide repeats in causative genes.
Tomoki Hirunagi, Kentaro Sahashi, Kiyoshi Tachikawa, Angel I. Leu, Michelle Nguyen, Rajesh Mukthavaram, Priya P. Karmali, Padmanabh Chivukula, Genki Tohnai, Madoka Iida, Kazunari Onodera, Manabu Ohyama, Yohei Okada, Hideyuki Okano, Masahisa Katsuno   +14 more
doaj   +1 more source

Polyglutamine Disease: Acetyltransferases Awry [PDF]

Current Biology, 2002
Recent evidence indicates that inhibition of histone acetyltransferases may be a primary cause of cellular pathogenesis in polyglutamine diseases such as Huntington disease; the results raise the possibility that pharmacologic manipulation of protein acetylation levels could be of therapeutic benefit.
openaire   +2 more sources

Ion channels and neuronal excitability in polyglutamine neurodegenerative diseases

Biomolecular Concepts, 2022
Polyglutamine (polyQ) diseases are a family composed of nine neurodegenerative inherited disorders (NDDs) caused by pathological expansions of cytosine-adenine-guanine (CAG) trinucleotide repeats which encode a polyQ tract in the corresponding proteins ...
Martinez-Rojas Vladimir A., Juarez-Hernandez Leon J., Musio Carlo   +2 more
doaj   +1 more source

Suppression of Mutant Protein Expression in SCA3 and SCA1 Mice Using a CAG Repeat-Targeting Antisense Oligonucleotide

Molecular Therapy: Nucleic Acids, 2019
Spinocerebellar ataxia type 3 (SCA3) and type 1 (SCA1) are dominantly inherited neurodegenerative disorders that are currently incurable. Both diseases are caused by a CAG-repeat expansion in exon 10 of the Ataxin-3 and exon 8 of the Ataxin-1 gene ...
Eleni Kourkouta, Rudie Weij, Anchel González-Barriga, Melissa Mulder, Ruurd Verheul, Sieto Bosgra, Bas Groenendaal, Jukka Puoliväli, Jussi Toivanen, Judith C.T. van Deutekom, Nicole A. Datson   +10 more
doaj   +1 more source

Fighting polyglutamine disease by wrestling with SUMO [PDF]

Journal of Clinical Investigation, 2015
Spinobulbar muscular atrophy (SBMA) is an X-linked disease characterized by degeneration of motor neurons, muscle atrophy, and progressive weakness. It is caused by a polyglutamine (polyQ) expansion in the androgen receptor (AR), a transcription factor that is activated upon hormone binding.
Craig, Tim J, Henley, Jeremy M
openaire   +3 more sources