Altered Metabolic Signaling and Potential Therapies in Polyglutamine Diseases [PDF]
MetabolitesPolyglutamine diseases comprise a cluster of genetic disorders involving neurodegeneration and movement disabilities. In polyglutamine diseases, the target proteins become aberrated due to polyglutamine repeat formation.
Alisha Vohra +2 more
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Skeletal Muscle Pathogenesis in Polyglutamine Diseases [PDF]
Cells, 2022 Polyglutamine diseases are characterized by selective dysfunction and degeneration of specific types of neurons in the central nervous system. In addition, nonneuronal cells can also be affected as a consequence of primary degeneration or due to neuronal
Caterina Marchioretti +5 more
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The Emerging Role of microRNAs in Polyglutamine Diseases [PDF]
Frontiers in Molecular Neuroscience, 2019 MicroRNAs (miRNAs) are small non-coding molecules that regulate a large amount of post-transcriptional repressor genes by recognizing semi-complementary target sequences that are normally located in the 3′ UTR of the mRNA. Altered expression of miRNA has
Xiaoyu Dong, Shuyan Cong
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Protein Aggregation Inhibitors as Disease-Modifying Therapies for Polyglutamine Diseases [PDF]
Frontiers in Neuroscience, 2021 The polyglutamine (polyQ) diseases are a group of inherited neurodegenerative diseases caused by the abnormal expansion of a CAG trinucleotide repeat that are translated into an expanded polyQ stretch in the disease-causative proteins. The expanded polyQ
Eiko N. Minakawa +2 more
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Olfactory dysfunction as an early pathogenic indicator in C. elegans models of Alzheimer's and polyglutamine diseases [PDF]
Frontiers in Aging NeuroscienceNeurodegenerative diseases such as Alzheimer's disease and polyglutamine diseases are characterized by abnormal accumulation of misfolded proteins, leading to neuronal dysfunction and subsequent neuron death.
Weikang Xue +8 more
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Protein Misfolding and Aggregation as a Therapeutic Target for Polyglutamine Diseases [PDF]
Brain Sciences, 2017 The polyglutamine (polyQ) diseases, such as Huntington’s disease and several types of spinocerebellar ataxias, are a group of inherited neurodegenerative diseases that are caused by an abnormal expansion of the polyQ tract in disease-causative proteins ...
Toshihide Takeuchi, Yoshitaka Nagai
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Advances in Modeling Polyglutamine Diseases Using Genome Editing Tools [PDF]
Cells, 2022 Polyglutamine (polyQ) diseases, including Huntington’s disease, are a group of late-onset progressive neurological disorders caused by CAG repeat expansions.
Marianna Karwacka, Marta Olejniczak
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Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3 [PDF]
Neurobiology of Disease, 2015 Accumulation of mutant polyglutamine proteins in intraneuronal inclusions is a hallmark of polyglutamine diseases. Impairment of protein clearance systems and sequestration of clearance-related proteins into inclusions occur in many protein folding ...
Li Zeng +9 more
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Pathogenesis of SCA3 and implications for other polyglutamine diseases [PDF]
Neurobiology of Disease, 2020 Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine (polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective disease genes. The nine known polyQ disease include Huntington’s disease (HD),
Hayley S. McLoughlin +2 more
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Nuclear accumulation of polyglutamine disease proteins and neuropathology [PDF]
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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