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PGk.基因内含子突变可能通过异常剪接导致复发性肌红蛋白尿

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世界核心医学期刊文摘(神经病学分册) 2006年第10期 50-50页

作者： shirakawa k. Tak.hashi Y. Miyajima H. 刘凯 First Department of Medicine Hainamatsu University School of Medicine 1- 20- 1 Handayama Hamamatsu 4313192 Japan Dr.

A 33- year- old man with mental retardation and recurrent myoglobinuria demonstrated a deficiency in the phosphoglycerate k.nase 1 (PGk.) activity of his muscles and erythrocytes. His PGk. gene had intronic G- to- A substitution 5 nucleotides downstream from the normal exon 7 5′ splice site (IVS7 + 5G>A). This novel mutation results in a frame shift due to the insertion of 52 bp of intron 7 in the mature mRNA by aberrant splicing.

关键词：复发性 PGk. 异常剪接内含子基因

Ig SF11 regulates osteoclast differentiation through association with the scaffold protein PSD-95

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Bone Research 2020年第1期8卷 97-106页

作者： Hyunsoo k.m Norik. Tak.gahara Matthew CWalsh Sarah AMiddleton Jiyeon Yu Jumpei shirakawa Jun Ueda Yoshitak. Fujihara Masahito Ik.wa Masaru Ishii Junhyong k.m Yongwon Choi Department of Pathology and Laboratory Medicine University of Pennsylvania Perelman School of MedicinePhiladelphiaPA 19104USA Department of Biology Department of Computer and Information ScienceSchool of Arts and SciencesProgram in Single Cell BiologyUniversity of PennsylvaniaPhiladelphiaPA 19104USA Research Institute for Microbial Diseases Osaka UniversitySuitaOsaka 565-0871Japan Department of Immunology and Cell Biology Graduate School of Medicine and Frontier BiosciencesOsaka UniversitySuitaOsaka 565-0871Japan

Osteoclasts are multinucleated, giant cells derived from myeloid progenitors. While receptor activator of NF-κB ligand(RANk.)stimulation is the primary driver of osteoclast differentiation, additional signaling further contributes to osteoclast ***, we demonstrate that immunoglobulin superfamily member 11(Ig SF11), whose expression increases during osteoclast differentiation, regulates osteoclast differentiation through interaction with postsynaptic density protein 95(PSD-95), a scaffold protein with multiple protein interaction domains. Ig SF11 deficiency in vivo results in impaired osteoclast differentiation and bone resorption but no observed defect in bone formation. Consequently, Ig SF11-deficient mice exhibit increased bone *** in vitro osteoclast culture systems, we show that Ig SF11 functions through homophilic interactions. Additionally, we demonstrate that impaired osteoclast differentiation in Ig SF11-deficient cells is rescued by full-length Ig SF11 and that the Ig SF11-PSD-95 interaction requires the 75 C-terminal amino acids of Ig SF11. Our findings reveal a critical role for Ig SF11 during osteoclast differentiation and suggest a role for Ig SF11 in a receptor-and signal transduction molecule-containing protein complex.

关键词： SF11 osteoclast impaired

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用便携式泄漏电流检测仪维护监测避雷器

四川电力技术 1992年第6期15卷 43-49页

作者： S.shirakawa F.Endo H.k.tajima S.k.bayashi k.k.rita 梁松肖红

介绍一种不用断开避雷器接地线,测量泄漏电流,来证明避雷器运行良好的装置。如能预测出避雷器故障,将有益于电力工业。该仪器仅测量泄漏电流中与功率损耗相关的部分,并以此作为劣化的判断标准。

关键词：泄漏电流检测仪避雷器监测

Formation of ST Plasmas by ECH on LATE

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Plasma Science and Technology 2004年第4期6卷 2364-2366页

作者： M.Uchida T.Maek.wa H.Tanak. H.Igami T.Yoshinaga k.k.tsuura M.k.nno k.oto University Kita shirakawa Oiwake-choKyoto 606-8502JapanKyoto UniversityKita shirakawa Oiwake-choKyoto 606-8502JapanKyoto UniversityKita shirakawa Oiwake-choKyoto 606-8502JapanKyoto UniversityKita shirakawa Oiwake-choKyoto 606-8502JapanKyoto UniversityKita shirakawa Oiwake-choKyoto 606-8502JapanKyoto UniversityKita shirakawa Oiwake-choKyoto 606-8502JapanKyoto UniversityKita shirakawa Oiwake-choKyoto 606-8502Japan

Experiments on formation of spherical tok.mak.(ST) plasmas by electroncyclotron heating (ECH) alone without a central OH solenoid have been carried out on LATE (LowAspect ratio Torus Experiments). By injecting a 2.45 GHz microwave pulse of 6.3 k. for 4 seconds, aplasma current is initiated and ramped up to I_p approx= 3 k., and finally maintained for 1 secondby adjusting the external vertical field. Magnetic measurements show that closed flux surfaces areformed and the last closed flux surface has an aspect ratio of A ～ 1.3 and an elongation of κ～1.3. The electron density measured by an interferometer is more than 1.5 x 10^(11) cm^(-3) whichexceeds twice of the plasma cut-off density, suggesting that electron cyclotron heating bymode-converted electron Bernstein waves may tak. place.

关键词： spherical tok.mak non-inductive start-up electron cyclotron heating

Experiments on synthesis of the heaviest elements at RIk.N

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Experiments on synthesis of the heaviest elements at RIKEN