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- 저자 : Tsukaguchi, H. (검색결과 2 건)
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Miyake, N.,Tsukaguchi, H.,Koshimizu, E.,Shono, A.,Matsunaga, S.,Shiina, M.,Mimura, Y.,Imamura, S.,Hirose, T.,Okudela, K.,Nozu, K.,Akioka, Y.,Hattori, M.,Yoshikawa, N.,Kitamura, A.,Cheong, H.,Kagami, S University of Chicago Press [etc.] 2015 American journal of human genetics Vol.97 No.4
The nuclear pore complex (NPC) is a huge protein complex embedded in the nuclear envelope. It has central functions in nucleocytoplasmic transport, nuclear framework, and gene regulation. Nucleoporin 107 kDa (NUP107) is a component of the NPC central scaffold and is an essential protein in all eukaryotic cells. Here, we report on biallelic NUP107 mutations in nine affected individuals who are from five unrelated families and show early-onset steroid-resistant nephrotic syndrome (SRNS). These individuals have pathologically focal segmental glomerulosclerosis, a condition that leads to end-stage renal disease with high frequency. NUP107 is ubiquitously expressed, including in glomerular podocytes. Three of four NUP107 mutations detected in the affected individuals hamper NUP107 binding to NUP133 (nucleoporin 133 kDa) and NUP107 incorporation into NPCs in vitro. Zebrafish with nup107 knockdown generated by morpholino oligonucleotides displayed hypoplastic glomerulus structures and abnormal podocyte foot processes, thereby mimicking the pathological changes seen in the kidneys of the SRNS individuals with NUP107 mutations. Considering the unique properties of the podocyte (highly differentiated foot-process architecture and slit membrane and the inability to regenerate), we propose a ''podocyte-injury model'' as the pathomechanism for SRNS due to biallelic NUP107 mutations.
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Impact of urbanization on user expectations related to public transport accessibility
Upali Vandebona,Hiroshi Tsukaguchi 서울시립대학교 도시과학연구원 2013 도시과학국제저널 Vol.17 No.2
An analysis of experience and expectations of urban residents towards accessibility to public transport systems from a number of cities at varying levels of urbanization is presented. A framework suitable to investigate differences of walking time to public transport facilities was initially established. It is then demonstrated that there is a relationship between the level of service of accessibility to public transport systems in a given urban area and expectations of its residents. Access to bus stops as well as railway stations has been considered. The paper provides a framework for planners to identify the perceived value of improvements to accessibility by accounting for the difference between the improvement of a physical measure of accessibility and the increased level of expectation of the subject community. The analysis has shown that it is important to keep the walking time for access to public transport systems below a specific value to ensure that the level of service associated with accessibility is within community expectations.
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