Results 181 to 190 of about 21,037 (206)
Polyglutamine protein aggregates are dynamic [PDF]
Nature Cell Biology, 2002 Protein aggregation and the formation of inclusion bodies are hallmarks of the cytopathology of neurodegenerative diseases, including Huntington's disease, Amyotropic lateral sclerosis, Parkinson's disease and Alzheimer's disease. The cellular toxicity associated with protein aggregates has been suggested to result from the sequestration of essential ...
Ellen A. A. Nollen +4 more
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Cell death in polyglutamine diseases
Cell and Tissue Research, 2000An increasing number of inherited neurodegenerative diseases are known to be caused by trinucleotide repeat expansions in the respective genes. At least nine disorders result from a CAG trinucleotide repeat expansion which is translated into a polyglutamine stretch in the respective proteins: Huntington's disease (HD), dentatorubral pallidolysian ...
Ullrich Wüllner +2 more
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pathogenesis of polyglutamine diseases
2010Polyglutamine (polyQ) diseases are neurodegenerative disorders caused by expansion in specific genes of a trinucleotide repeat, cytosine–adenine–guanine (CAG), which encodes glutamine. In 1991, CAG repeat expansion in the androgen receptor was linked to spinal and bulbar muscular atrophy (SBMA), thus indicating for the first time a causative role of ...
Pennuto, Maria, Sambataro, Fabio
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Polyglutamine Diseases and Molecular Chaperones [PDF]
IUBMB Life, 2003 AbstractThe polyglutamine diseases, a group of diseases currently thought to consist of nine inherited neurodegenerative diseases, are caused by the expansion of unstable CAG trinucleotide repeats that code for polyglutamine tracts in the responsible genes.
Akira Kakizuka, Yoko Kimura
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Polyglutamine makes the switch
Science Signaling, 2017In worms, a regulator of noncoding RNA directly catalyzes formation of toxic protein aggregates in the presence of polyglutamine.
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[MicroRNAs and polyglutamine diseases].
Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2013Polyglutamine(PolyQ) diseases comprise a group of inherited neurodegenerative disorders with significant clinical and genetic heterogeneity. Although they share a common mechanism involving dynamic expansion of CAG trinucleotide repeats, their clinical features may vary and there has been no specific treatment.
Hong Jiang, Li Zhang
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The role of chaperones in polyglutamine disease
Trends in Molecular Medicine, 2002The "triplet repeat" neurodegenerative diseases are linked by a common mode of pathogenicity, wherein a polyglutamine expansion within the relevant disease-causing protein induces toxicity. Although details underlying pathogenesis are largely unknown, protein chaperones appear to be effective suppressors of toxicity in various experimental models ...
Huda Y. Zoghbi, Puneet Opal
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Molecular Pathways to Polyglutamine Aggregation
2012Over 100 human cellular proteins contain a repetitive polyglutamine tract, however, only nine ofthese proteins are associated with disease. In these proteins, the expanded polyQ tract perturbs the native conformation, resulting in an ordered aggregation process that leads to the formation of amyloid-like fibrils.
Amy L. Robertson, Stephen P. Bottomley
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Finding a role for polyglutamine
C&EN Global Enterprise, 2017In nine neurodegenerative diseases, the culprit is an increase in the number of repeated glutamines in a certain protein. For example, healthy people have between six and 35 glutamines in a row in their huntingtin proteins, whereas patients with Huntington’s disease can have more than 100.
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Monitoring polyglutamine toxicity in yeast
Methods, 2011Experiments in yeast have significantly contributed to our understanding of general aspects of biochemistry, genetics, and cell biology. Yeast models have also delivered deep insights in to the molecular mechanism underpinning human diseases, including neurodegenerative diseases.
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