Results 51 to 60 of about 14,352 (184)
The Missing Link in Polyglutamine Diseases [PDF]
Movement Disorders Clinical Practice, 2017 Polyglutamine diseases are a group of nine hereditary neurodegenerative disorders and include Huntington disease (HD), the most prevalent spinocerebellar ataxias (SCAs type 1, 2, 3, 6, 7 and 17), dentatorubral-pallidoluysian atrophy and spinal and bulbar muscular atrophy (also known as Kennedy disease).
Aziz, N. Ahmad, Balint, Bettina
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Molecules, 2014 Polyglutamine (polyQ) aggregation plays a pivotal role in the pathological process of Huntington’s disease and other polyQ disorders. Therefore, strategies aiming at restoring dysfunction and reducing stresses mediated by polyQ toxicity are of ...
Lingyun Xiao +9 more
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Hunting for the cause: Evidence for prion-like mechanisms in Huntington’s disease
Frontiers in Neuroscience, 2022 The hypothesis that pathogenic protein aggregates associated with neurodegenerative diseases spread from cell-to-cell in the brain in a manner akin to infectious prions has gained substantial momentum due to an explosion of research in the past 10–15 ...
Kirby M. Donnelly +7 more
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Stimulating proteasomal degradation in human proteinopathies
The FEBS Journal, EarlyView.The proteasome mediates the degradation of a wide range of proteins. Boosting proteasomal degradation may be beneficial in several disease contexts and can be achieved either by modulating proteasome activity or by improving substrate delivery. Proteasome activity can be enhanced by increasing proteasome abundance, inducing constitutive gate opening ...
Maria E. Gierisch +2 more
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Stem cell models of polyglutamine diseases and their use in cell-based therapies
Frontiers in Neuroscience, 2015 Polyglutamine diseases are fatal neurological disorders that affect the central nervous system. They are caused by mutations in disease genes that contain CAG trinucleotide expansions in their coding regions.
Evangelia eSiska +3 more
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Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3
Cells, 2022 Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder showing progressive neuronal loss in several brain areas and a broad spectrum of motor and non-motor symptoms, including ataxia and altered sleep.
Maria-Efstratia Tsimpanouli +6 more
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Cell Death in Neurodegenerative Diseases: Molecular Mechanisms and Therapeutic Targets
MedComm, Volume 7, Issue 9, September 2026.Regulated cell death pathways, including apoptosis, necroptosis, pyroptosis, ferroptosis, and autophagy‐dependent cell death, interact with mitochondrial dysfunction, proteostasis failure, lysosomal stress, glial remodeling, and neuroinflammation across major neurodegenerative diseases.
Tianjiao Li +3 more
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Frontiers in Cell and Developmental Biology, 2022 Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited cerebellar ataxia caused by the expansion of a polyglutamine (polyQ) repeat in the gene encoding ATXN3.
Karen Jansen-West +22 more
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Technologies for engineering repetitive DNA
Quantitative Biology, Volume 14, Issue 3, September 2026.Abstract Repetitive DNA, a fundamental architectural element of genomes, is widespread across organisms and comprises about 54% of the human genome. With advances in long‐read sequencing and bioinformatics approaches, highly repetitive sequences can now be characterized in depth.
Shuting Ma, Yali Cui, Yi Wu
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Polyglutamine inclusion body toxicity [PDF]
Movement Disorders, 2017 Peer Reviewed ; https://deepblue.lib.umich.edu/bitstream/2027.42/141741/1/mds27226_am.pdf ; https://deepblue.lib.umich.edu/bitstream/2027.42/141741/2/mds27226 ...
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