No abstract

Huntington's disease (HD) is a genetic and neurodegenerative disease, leading to motor and cognitive dysfunction in HD patients. At cellular level, this disease is caused by the accumulation of mutant huntingtin (HTT) in different cells, and finally results in the dysfunction of different cells. To clean these mutant proteins, ubiquitin-proteasome system (UPS) and autophagy system are two critical pathways in the brain; however, little is known in other peripheral tissues. As mutant HTT affects different tissues progressively and might influence the UPS and autophagy pathways at early stages, we attempted to examine two clearance systems in HD models before the onset. Here, in vitro results showed that the accumulation of UPS signals with time was observed obviously in neuroblastoma and kidney cells, not in other cells. In HD transgenic mice, we observed the impairment of UPS, but not autophagy, over time in the cortex and striatum. In heart and muscle tissues, disturbance of autophagy was observed, whereas dysfunction of UPS was displayed in liver and lung. These results suggest that two protein clearance pathways are disturbed differentially in different tissues before the onset of HD, and enhancement of protein clearance at early stages might provide a potential stratagem to alleviate the progression of HD.

To develop the numerical control program of mill-turn machine, the traditional method is to apply the computer-aided design and manufacture software to construct the geometric model, then to generate tool path and convert the path to NC program. For complex numerical control program of mill-turn machine, such as the multiple turret synchronized motion machining, because of the need to control time sequence, the NC program is highly required on using of dedicated software system. The objective of this paper is to establish a mill-turn machining system with window interface of via the language of Borland C++ Builder. The developed system can plan the machining path of simple mill-turn features, including turning shape, axial slot milling, and radial packet milling, and generate the corresponding NC program. For the milling functions, after the offset coordinates are calculated along the polygonal angle vector in the center point of cutters, the NC program is generated. For the turning functions, through importing the 2D DXF (Drawing Exchange Format) file and inputting related configurations, the entity coordinates can be retrieved and the corresponding NC program is then converted. By means of the solid cutting simulation software and practical cutting experiment for the generated numerical control program, the accuracy of the tool path generation algorithm is confirmed. Hence, the cost of purchasing commercial software can be saved and the time of generating program can also be decreased so that the working efficiency can be enhanced.

Resides derived from liquefaction of oil palm trunk in the presence of polyhydric alcohol with different liquefaction temperature and time were characterized to provide a new approach to understand some fundamental aspects of the liquefaction reaction. Higher temperature and longer reaction time resulted in lower residue content, indicating more decomposition of components of oil palm trunk. The amorphous polymer comprised of lignin, hemicellulose, starch, and cellulose with non-crystalline structure are firstly degraded at low liquefaction temperature, followed by the decomposition of crystalline region of cellulose. Although it was relatively difficult to destroy the ordered structure of cellulose, most of them could be liquefied via prolonging reaction time or enhancing reaction temperature. Nevertheless, it was found that re-condensation of liquefied products occurred during the liquefaction process when higher temperature of 180 oC was used after 60 min, leading to the gradual increase of residue content with increase of reaction time.