9 research outputs found
Identification of Two Phenanthrene Derivatives from Australasian Allied Species in Genus <i>Dendrobium</i>
Biallelic Mutations in Nuclear Pore Complex Subunit NUP107 Cause Early-Childhood-Onset Steroid-Resistant Nephrotic Syndrome
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
幼稚園教育のいくつかの課題 : 「幼稚園教育が直面する複合課題に関する共同的研究」まとめとして
幼稚園教育がかかえる複合的な課題を検討する目的で,1幼稚園を対象に共同研究を実施した。以下のような課題別に共同研究者は研究を分担した。今日的な幼稚園教育の意義を確認する意図で子どもの主体性尊重の保育を,また障害幼児や外国人幼児を含む保育の意義を検討する意図で,その具体的な保育を,さらに, 子どもや家族に幼稚園教育が果たす意義を検討する意図で,保護者からボ唆される課題について研究を実施した。幼稚園教育に寄与する諸点を見いたした。To investigate several issues on kindergarten education, we conducted collaborative research based on three themes: Child-centered education and the purpose of kindergarten education today, the concrete educational examples of children with disabilities and children from foreign counties and what education means for them, and the problems of kindergarten in the view of the parents. As a result, some findings were made which will be useful for the future development of kindergarten education.11KJ0000870999