23 research outputs found

    Dicer Is Required for Maintaining Adult Pancreas

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    Dicer1, an essential component of RNA interference and the microRNA pathway, has many important roles in the morphogenesis of developing tissues. Dicer1 null mice have been reported to die at E7.5; therefore it is impossible to study its function in adult tissues. We previously reported that Dicer1-hypomorphic mice, whose Dicer1 expression was reduced to 20% in all tissues, were unexpectedly viable. Here we analyzed these mice to ascertain whether the down-regulation of Dicer1 expression has any influence on adult tissues. Interestingly, all tissues of adult (8–10 week old) Dicer1-hypomorphic mice were histologically normal except for the pancreas, whose development was normal at the fetal and neonatal stages; however, morphologic abnormalities in Dicer1-hypomorphic mice were detected after 4 weeks of age. This suggested that Dicer1 is important for maintaining the adult pancreas

    Dicer Is Required for Maintaining Adult Pancreas

    Get PDF
    Dicer1, an essential component of RNA interference and the microRNA pathway, has many important roles in the morphogenesis of developing tissues. Dicer1 null mice have been reported to die at E7.5; therefore it is impossible to study its function in adult tissues. We previously reported that Dicer1-hypomorphic mice, whose Dicer1 expression was reduced to 20% in all tissues, were unexpectedly viable. Here we analyzed these mice to ascertain whether the down-regulation of Dicer1 expression has any influence on adult tissues. Interestingly, all tissues of adult (8–10 week old) Dicer1-hypomorphic mice were histologically normal except for the pancreas, whose development was normal at the fetal and neonatal stages; however, morphologic abnormalities in Dicer1-hypomorphic mice were detected after 4 weeks of age. This suggested that Dicer1 is important for maintaining the adult pancreas

    Single nucleotide variations in CLCN6 identified in patients with benign partial epilepsies in infancy and/or febrile seizures

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    Nucleotide alterations in the gene encoding proline-rich transmembrane protein 2 (PRRT2) have been identified in most patients with benign partial epilepsies in infancy (BPEI)/benign familial infantile epilepsy (BFIE). However, not all patients harbor these PRRT2 mutations, indicating the involvement of genes other than PRRT2. In this study, we performed whole exome sequencing analysis for a large family affected with PRRT2-unrelated BPEI. We identified a non-synonymous single nucleotide variation (SNV) in the voltage-sensitive chloride channel 6 gene (CLCN6). A cohort study of 48 BPEI patients without PRRT2 mutations revealed a different CLCN6 SNV in a patient, his sibling and his father who had a history of febrile seizures (FS) but not BPEI. Another study of 48 patients with FS identified an additional SNV in CLCN6. Chloride channels (CLCs) are involved in a multitude of physiologic processes and some members of the CLC family have been linked to inherited diseases. However, a phenotypic correlation has not been confirmed for CLCN6. Although we could not detect significant biological effects linked to the identified CLCN6 SNVs, further studies should investigate potential CLCN6 variants that may underlie the genetic susceptibility to convulsive disorders.Toshiyuki Yamamoto, Keiko Shimojima, Noriko Sangu, Yuta Komoike, Atsushi Ishii, Shinpei Abe, Shintaro Yamashita, Katsumi Imai, Tetsuo Kubota, Tatsuya Fukasawa, Tohru Okanishi, Hideo Enoki, Takuya Tanabe, Akira Saito, Toru Furukawa, Toshiaki Shimizu, Carol J. Milligan, Steven Petrou, Sarah E. Heron, Leanne M. Dibbens, Shinichi Hirose, Akihisa Okumur

    Commerce et contrebande des indiennes en Provence dans la deuxième moitié du XVIIIe siècle.

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    Das Register der Intendanz der Provence enthält wertvolle Dokumente, die das Ausmass des manchmal im Verborgenen stattfindenden Handels mit Indienne-Kattun nach der Aufhebung des Verbots von 1759 offenlegen. Man entdetckt nicht nur die Struktur eines regionalen Handels, sondern auch die sonderbare Beziehung zwischen den Herstellern der Kattunstoffe und dem Schmuggel so wie die Existenz einer von der hugenottischen Diaspora organisierten « Internationale des Indiennes » (Binnenhandel und Seeverkehr).The archives of Provence's Intendancy contain precious documents that reveal the breadth of the often clandestine chintz trade after the lifting of the prohibition in 1759. One discovers not only the structures of regional trade but also the curious relationships between chintz manufacturers and smuggling as well as the existence of a « Chintz international » that was organized by the Huguenot diaspora (domestic and maritime trade).Le fonds de l'Intendance de Provence contient de précieux documents qui révèlent l'ampleur du commerce, parfois clandestin, des indiennes après la levée de la prohibition en 1759. On y découvre non seulement la structure d'un commerce régional, mais les curieux rapports entre les fabricants d'indiennes et la contrebande, ainsi que la présence d'une « Internationale des indiennes » organisée par la diaspora huguenote (commerce intérieur et trafic maritime).Fukasawa Katsumi. Commerce et contrebande des indiennes en Provence dans la deuxième moitié du XVIIIe siècle.. In: Annales du Midi : revue archéologique, historique et philologique de la France méridionale, Tome 99, N°178, 1987. Enfants abandonnés enfants emprisonnés, les méridionaux et l'Orient. pp. 175-192

    L'acceptation et l'exclusion de l'autre dans l'histoire française moderne

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    La question de 1’autre, quelle qu’en soit la définition, attire de plus en plus les historiens, pour une raison évidente : l’évolution du monde contemporain, où se multiplient partout les affrontements religieux, culturels, ethniques ou « nationaux », au point qu’on parle même, à tort sans doute, de « choc des civilisations ». Ainsi, des barbaroi et des xénoi de l’antiquité grecque aux réfugiés russes comme aux immigrés maghrébins et indochinois du « creuset français » du XXe siècle, la prése..

    18世紀のフランス=レヴァント貿易と国際金融(下) : ルー商会文書の為替手形

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    Ⅳ.レヴァントの商人と手形振出経路 / V.解釈と考察の試み / 結

    ルー商会文書の為替手形 : 18世紀金融技術の基礎研究

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    序 / I.史料 / II.為替手形の形態と構成 / III.裏書と割引 / 結

    18世紀のフランス=レヴァント貿易と国際金融(上) : ルー商会文書の為替手形

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    序 / Ⅰ 史料と研究対象 / Ⅱ 為替手形流通の空間的構造 / Ⅲ ルー商会と手形引渡経
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