منابع مشابه
Modeling polyglutamine pathogenesis in C. elegans.
A growing number of human neurodegenerative diseases are associated with disruption of cellular protein folding homeostasis, leading to the appearance of misfolded proteins and deposition of protein aggregates and inclusions. Recent years have been witness to widespread development of invertebrate systems (specifically Drosophila and Caenorhabditis elegans) to model these disorders, bringing th...
متن کاملPolyglutamine Diseases-Understanding the Mechanism of Pathogenesis
Protein misfolding has been implicated in a large number of diseases, which are now grouped under the name of Protein conformational disorders (PCDs). Few examples of diseases that fall in this group are Alzheimer’s disease, Parkinson’s disease and Huntington’s disease. All these disorders are characterized by sets of protein that misfold and aggregate in specific tissues. In order to identify ...
متن کاملPathogenesis of polyglutamine disorders: aggregation revisited.
Expansion of CAG trinucleotide repeats coding for polyglutamine in unrelated proteins causes at least nine late-onset progressive neurodegenerative disorders, including Huntington's disease and a number of spinocerebellar ataxias. Expanded polyglutamine provokes a dominant gain-of-function neurotoxicity, regardless of the specific protein context within which it resides. Nevertheless, the prote...
متن کاملInactivation of Drosophila Apaf-1 related killer suppresses formation of polyglutamine aggregates and blocks polyglutamine pathogenesis.
Huntington's disease (HD) is caused by expansion of a polyglutamine tract near the N-terminal of huntingtin. Mutant huntingtin forms aggregates in striatum and cortex, where extensive cell death occurs. We used a Drosophila polyglutamine peptide model to assess the role of specific cell death regulators in polyglutamine-induced cell death. Here, we report that polyglutamine-induced cell death w...
متن کاملPolyglutamine diseases: emerging concepts in pathogenesis and therapy.
Polyglutamine diseases are a family of neurodegenerative conditions that each derive from a CAG triplet repeat expansion in a specific gene. This produces a pathogenic protein that contains a critically expanded tract of glutamines. These prototypical protein misfolding disorders include Huntington disease, spinobulbar muscular atrophy, dentatorubral-pallidoluysian atrophy and several spinocere...
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ژورنال
عنوان ژورنال: Neuron
سال: 2002
ISSN: 0896-6273
DOI: 10.1016/s0896-6273(02)00872-3