Sentence examples for modifiers of polyglutamine from inspiring English sources

We recognized this fact could directly influence the aggregation kinetics of huntingtin, since studies have shown that destabilized proteins may function as non-specific modifiers of polyglutamine misfolding by sequestering chaperones and eliciting global protein folding stress [45].

A genome-wide RNAi screen for modifiers of polyglutamine aggregation identified close to 200 modifiers.

Altogether, these findings have provided explanations for the specificity of RNA metabolism genes as modifiers of polyglutamine toxicity.

For over 10 years, DNAJ (HSP40) chaperones have been recognized as potent modifiers of polyglutamine aggregation and toxicity (Muchowski and Wacker, 2005).

Another example of a functional gene class that is specifically found for one type of neurodegenerative disease-related proteins are the many RNA-processing components as modifiers of polyglutamine toxicity and aggregation.

Interestingly, over-expression of HSP40 chaperones has been reported to suppress polyglutamine aggregation more potently than HSP70 chaperones, with members of a DNAJB subfamily of HSP40 molecules proving to be the most powerful modifiers of polyglutamine aggregation in cell culture (Hageman et al., 2010).

Larry Marsh used a Drosophila model of Huntington's disease (HD) to screen for modifiers of polyglutamine-induced neurotoxicity and identified sir2 (Drosophila homolog of SIRT1) and sirt2.

These findings confirm that HSJ1a is a potent modifier of polyglutamine aggregation and, importantly, demonstrate that previous observations in cell culture translate to the mouse brain (Westhoff et al., 2005; Borrell-Pages et al., 2006; Howarth et al., 2007; Hageman et al., 2010).

Using libraries of genetically altered transposon (P-element) insertion strains, additional modifiers of toxicity of polyglutamine proteins have been screened for.

Although there is an inherent level of variability in gene expression, this method has proved powerful for the study of disease modifiers in models of polyglutamine disease [14] and motor neuron disease [16 19] and in the evaluation of therapeutic strategies [15].

However, given that so many studies use the eye for validation of polyglutamine modifiers, it should be noted that perhaps not all such modifiers will prove valid in models for mutant Htt aggregation.