Not exact matches
This abnormal
binding causes more VCP accumulation on the mitochondria,» Nerve cells with VCP - mutant
huntingtin interacting inside them became dysfunctional and self - destructed.
«Because mitochondrial dysfunction has been proposed to play an important role in the pathogenesis of Huntington's disease,» said Qi, «we investigated the
binding proteins of mutant
huntingtin on mitochondria.»
«We already know from previous studies on the protein
huntingtin, which spontaneously forms aggregates and is responsible for the development of the neurodegenerative disorder Huntington's disease, that protein aggregates also
bind to essential proteins that have no defects.»
Yano and her colleagues found that the mutated
huntingtin protein
binds to a group of proteins called TIM23.
In an earlier study, Subramaniam and his colleagues showed that Rhes
binds to a series of repeats in the
huntingtin protein (named for its association with Huntington's disease), increasing the death of neurons.
Huntingtin null neurons exhibit a significant reduction in transcripts encoding proteins destined for the extracellular space, many of which are components of the extracellular matrix or involved in cellular adhesion, receptor
binding and hormone activity.