Exact(1)
These pathological features somewhat resemble those observed in the skeletal muscle of DM1 mice, which expressed expanded CUG transcripts [13], [14].
Similar(59)
Later during cellular differentiation events, the compartments where JMJ is expressed expand gradually, with expression detectable in almost all adult tissues, although the intensities vary among cell types., These observations suggest that histone demethylases link core transcription factor networks to the regulation of chromatin status during cellular differentiation.
Ubb was amongst the genes with increased expression in cells expressing expanded polyQ containing TBP.
Haeusler et al. [ 11] observed disruption of nucleolin expression from the nucleolus in cell models expressing expanded C9ORF72, but reported a variable distribution or nucleolin in C9ORF72+ CNS tissue.
FCS performed on lysates of cells expressing expanded polyQ-GFP also detected formation of soluble oligomers [30].
To address the possibility that this increased aggregation of 2UIM ataxin-3 reflects transient overexpression in nonneuronal cells, we compared levels of SDS-insoluble ataxin-3 aggregates in whole brain lysates from aged MJD84.2 mice, which express primarily expanded Q84 3UIM ataxin-3 transprotein, and Q71B mice, which only express expanded Q71 2UIM ataxin-3 transprotein (Figure 4C and 4D).
It has been shown to protect neurons from degeneration in the absence of any additional aggregated protein[10], [11] and improves the survival of animals expressing expanded polyglutamine proteins associated with Huntington disease[30].
For polyglutamine-containing protein detection, two stably transfected cell lines expressing expanded ataxin-3 proteins with 54 and 109 CAG repeats (a gift from Dr. Hsieh M). were used as positive controls.
Molecular hallmarks of cells expressing expanded repeat RNA are nuclear RNA foci of distinct morphology and abundance.
We further set out to determine whether GAPDH selectively recognizes and associates with mitochondria in cells expressing expanded polyglutamine repeats.
We found that expressing expanded CGG repeats in olfactory neurons interfered with this plasticity without affecting either the innate odor-seeking response or the olfactory neuronal morphology.
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