Three distinct OCRL1 mutations in three patients with the Dent disease phenotype are described. All the patients manifested an extremely high degree of low-molecular-weight proteinuria and showed no ocular abnormalities or apparent mental retardation. Urinalysis and blood chemistry showed no findings suggestive of Fanconi syndrome with renal tubular acidosis. Mutations in CLCN5 were ruled out. The mutations identified in OCRL1 are one frame-shift mutation (I127stop) and two missense mutations (R301C and R476W). R301C and R476W mutations might be hot spots in OCRL1, which develop very similar phenotypes as Dent-2.
In L2 acquisition, there has long been a debate as to whether instructors should conduct classes in English or through the approach of code-switching. This study was conducted to investigate the impact on learning anxiety in classes taught only in English. The survey was conducted with 55 students at a university in central Tokyo. A questionnaire and interviews were carried out to see how the students’ learning anxiety changed over a period of four months of instruction. The results showed that the students with prior experience of lessons conducted in English (LCE) had reduced learning anxiety over the period. Qualitative analysis was also conducted regarding the factors in students’ learning anxiety. The researchers argue that the results of this research can be used to enhance teacher efficacy through the sharing the effects of lessons conducted in English with instructors teaching English in Japan.
第二言語習得において教員の間では、オールイングリッシュあるいはコードスィッチングのどちらがより効果的に英語の指導ができるか長い間議論されている。本研究では、オールイングリッシュ指導がもたらす影響を論証する。東京都内の大学に在籍する大学生55名を対象にアンケートと個別面談を実施し、オールイングリッシュクラスを受講する学生の不安度が4ヶ月でどのように変化したかを分析した。その結果、大学入学前にオールイングリッシュクラスの受講経験を有する学生は、英語学習に対する不安度を有意に軽減させたことが分かった。また、学生が授業で感じた不安材料とそれに影響を与えた要素を考察した。その研究結果をもとに、オールイングリッシュ指導の有効性や今後教員間で効果的な指導のために共有できるものはなにかを提案し論じる。
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