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Matysiak-Budnik Tamara,Jamet Philippe,Chapelle Nicolas,Fabiani Bettina,Coppo Paul,Ruskoné-Fourmestraux Agnès 거트앤리버 소화기연관학회협의회 2022 Gut and Liver Vol.16 No.2
Background/Aims: Primary gastrointestinal follicular lymphomas (PGFL) are very rare. Our aim was to analyze the clinical features, management, and long-term outcomes in a prospective series of patients diagnosed with PGFL. Methods: All adult patients with PGFL, consecutively enrolled into the multicenter French study between 1990 and 2017, were evaluated and followed up prospectively after undergoing a complete work-up. Clinical, pathological and endoscopic features, as well as treatment outcomes, were analyzed. Results: Thirty-one patients (16 men, median age 62 years, range 33 to 79 years) with PGFL were included. The median follow-up was 92 months (range, 6 to 218 months). In the majority of patients (n=14), lymphoma was incidentally diagnosed during endoscopy. Otherwise, the most frequent circumstances of diagnosis were abdominal pain (n=7) and dyspepsia (n=5). The duodenum was the most common site of involvement (n=19) and multifocal localizations were seen in seven patients (22%). The most frequent first line strategy was surveillance applied in 22 patients (71%), of whom nine reached spontaneous, complete remission and 11 had stable disease. Seven patients (23%) received chemotherapy as first line treatment, and two underwent resection. Of seven patients who received chemotherapy, four achieved complete remission. In three patients, transformation into a high-grade lymphoma occurred. Conclusions: The diagnosis of PGFL is frequently fortuitous. The most common localization is in the duodenum. The disease has an indolent course and a good prognosis, however, rare cases of transformation into aggressive high-grade lymphoma may occur. An appropriate characterization and follow-up of these lymphomas is mandatory for their optimal management.
Thomas, Ulrich,Kim, Eunjoon,Kuhlendahl, Sven,Koh, Young Ho,Gundelfinger, Eckart D.,Sheng, Morgan,Gamer, Craig C.,Budnik, Vivian 부산대학교 유전공학연구소 1997 분자생물학 연구보 Vol.13 No.-
The cells adhesion molecule FasciclinⅡ(FASⅡ)is invoved in synapse development and plasticity. Here we provide genetic and biochemical evidence that proper localization of FASⅡ at typeⅠglutamatergic aynapses of the Drosophila neuromuscular junction is mediated by binding between the intracellular tSXV bearing C-terminal tail of FASⅡ and the PDZ1-2 domains of Discs-Large (DLG). Moreover, mutations in fasⅡ and /or dlg have similar effects on presynaptic ultrastructure, suggesting their functional involvement in a common developmental pathway. DLG can directly mediate a biochemical complex and a macroscopic cluster of Ⅱ and Shaker K^+ channels in heterologous cells. These results indicate a central role for DLG in the structural organization and downstream signaling mechanisms of cell adhesion molecules and ion channels at synapses.
Essential Role for dlg in Synaptic Clustering of Shaker K^+ Channels In Vivo
Tejedor, Francisco J.,Bokhari, Amr,Rogero, Oscar,Gorczyca, Michael,Zhang, Jiangwen,Kim, Eunjoon,Sheng, Morgan,Budnik, Vivian 부산대학교 유전공학연구소 1997 분자생물학 연구보 Vol.13 No.-
The assemblage of specific ion channels and repectors at synaptic sites is crucial for signaling betweem pre-and postsynaptic cells. However, the mechanism by which proteins are targeted to and cluatered at synapses are poorly understood. Here we show that the producct of the Drosophila discs-large gene, DLG, is colocalized with Shaker K^+ chammels, which are clustered at glutamatergic synapses at the larval neuromuscular juction. In heterologous cells. DLG can cluster Shaker-type K^+ channels,and in the yeast two-hybrid system,the DLG PDZ1-2 domains bind directly to the DLG-Shaker interactions are required in vivo for Shaker clustering at the neuromuscular junction. Synaptic cluatering of Shaker channels is abolished not only by mutations in dlg but also by a mutation in Shaker that deletes its C-terminal DLG binding motif. Analyses of various dlg mutant alleles suggest that channel clustering and synaptic targeting functions depend on distinct DLG domains. These studies demonstrate for the first time that DLG plats an important role in sunaptic organization in vivo that correlates with its ability to bind directly to specific menbrane proteins of the synapse.