Huntington disease (HD) is an inherited neurodegenerative disorder characterized by motor and cognitive dysfunction caused by expansion of polyglutamine (polyQ) repeat in exon 1 of huntingtin (HTT). In patients, the number of glutamine residues in polyQ tracts are over 35, and it is correlated with age of onset, severity, and disease progression. Expansion of polyQ increases the propensity for HTT protein aggregation, process known to be implicated in neurodegeneration. These pathological aggregates can be transmitted from neuron to another neuron, and this process may explain the pathological spreading of polyQ aggregates. Here, we developed an in vivo model for studying transmission of polyQ aggregates in a highly quantitative manner in real time. HTT exon 1 with expanded polyQ was fused with either N-terminal or C-terminal fragments of Venus fluorescence protein and expressed in pharyngeal muscles and associated neurons, respectively, of C. elegans. Transmission of polyQ proteins was detected using bimolecular fluorescence complementation (BiFC). Mutant polyQ (Q97) was transmitted much more efficiently than wild type polyQ (Q25) and forms numerous inclusion bodies as well. The transmission of Q97 was gradually increased with aging of animal. The animals with polyQ transmission exhibited degenerative phenotypes, such as nerve degeneration, impaired pharyngeal pumping behavior, and reduced life span. The C. elegans model presented here would be a useful in vivo model system for the study of polyQ aggregate propagation and might be applied to the screening of genetic and chemical modifiers of the propagation.
Robots for communication are developed extensively with emphasis on sympathy. This study deals with the growth of character and the control of its operation accordingly. The child has time to be alone with the nature of his/her robot friend. The child can interact with other people's emotional expressions through a robot. Step by step, the robot character will grow as the child grows. Through design studies, qualitative processes such as {customer experience audit, eye tracking, mental model diagrams, semantic differences} have been executed for the results. The participatory behavior research approach through user travel is mapped from the user's lead to the evidence-based design. This research considers how the synthetic characteristics can be applied to the physical growth of robot toys through the product design process. With the development of robot toy "Buddy", we tried making two variations on the robot to achieve recognizable growth. (1) An one-dimensional height scaling and (2) facial expression including the distance between two eyes on the screen. Observations represented children's reactions when "Buddy" was released to with the children. As an independent synthetic character, the robot was recognized by children who had the designed function. Robots for training may require more experimentation.
Robots for communication are developed extensively with an emphasis on sympathy. This study deals with the growth of character and the control of its operation. The child has time to be alone with the nature of his/her robot friend. That child can interact with other people’s emotional expressions through a robot. Step by step, the robot character will grow as the child grows. Through design studies, qualitative processes such as customer experience audit, eye tracking, mental model diagrams, and semantic differences have been executed for the results. The participatory behavior research approach through user travel is mapped from the user’s lead to the evidence-based design. This research considers how synthetic characteristics can be applied to the physical growth of robot toys through the product design process. With the development of robot toy “Buddy”, two variations on the robot were made to achieve recognizable growth. (1) one-dimensional height scaling and (2) facial expression including the distance between two eyes on the screen. Observations represented children’s reactions when "Buddy" was released with the children. As an independent synthetic character, the robot was recognized by children who had the designed function. Robots for training may require more experimentation.
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