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이범,허선희,전종근,김유미,김자혜,최인희,김재민,김구환,유한욱,Lee, Beom Hee,Heo, Sun H.,Cheon, Chong Kun,Kim, Yoo-Mi,Kim, Ja Hye,Choi, In Hee,Kim, Jae-Min,Kim, Gu-Hwan,Yoo, Han-Wook 대한유전성대사질환학회 2013 대한유전성대사질환학회지 Vol.13 No.1
Gaucher disease is a lysosomal storage disease caused by deficiency of glucocerebrosidase (GBA). This condition is characterized by accumulation of glucocerebrosidase in liver, spleen, lung, skeletal system, and central nervous system. Gaucher disease is the prototype of disease in which efficacy of enzyme replacement therapy has been established. However, because recombinant enzyme is not able to enter the central nervous system, its efficacy is limited to the non-neurological manifestations of Gaucher disease. Importantly, approximately a half of Korean patients with Gaucher disease suffer from neurological manifestations. In addition, Korean Gaucher disease patients exhibit distinct mutation spectrum from those in other populations. Common mutations in Korean patients with Gaucher disease are also associated with neurological phenotype. Therefore, therapeutic strategies tailored to Korean patients were necessary. Interestingly, a chemical chaperone, ambroxol, has been known to increase residual enzymatic activities of the select mutant GBAs encoded by mutations prevalent in Korean patients. One promising aspect of this drug is that it can cross blood-brain barrier, and enhance the enzyme activity in the brain. In vitro study suggested this chemical chaperone as one of new therapeutic agents in Gaucher disease, and a well-designed human trial is required to confirm its efficacy.
수학 성취수준 진단을 위한 준거 설정 방법에 대한 고찰
도종훈(Do Jong Hoon),허선희(Heo Sun Hee) 서원대학교 사범대학 부설 교육연구소 2015 교육발전 Vol.35 No.1
In this paper we review several theories and criteria to evaluate levels of mathematics achievement, especially a modified Angoff method which has been used for determining students achievement levels in the National Assessment of Educational Achievement of Mathematics, and then discuss how to compare students mathematics achievement and differentiate classes in each school.
중학교 수학 ① 스토리텔링 모델교과서 개발 및 적용 연구
이재학 ( Jae Hak Lee ),도종훈 ( Jong Hoon Do ),박윤범 ( Yun Beom Park ),박혜숙 ( Hye Sook Park ),신준국 ( Joon Kook Shin ),김정자 ( Jeong Ja Kim ),허선희 ( Sun Hee Heo ) 한국수학교육학회 2013 수학교육논문집 Vol.27 No.3
본 연구에서는 중학교 수학 ① 수업에 적용 가능한 스토리텔링 모델교과서의 예시 단원을 개발한다. 그리고 개발된스토리텔링 모델교과서를 활용한 실험 수업을 실시하고, 수업 지도 교사와의 면담 및 학생들에 대한 설문 조사를 통해 스토리텔링 모델교과서를 활용한 수업에서 드러나는 어려움 및 장점 등을 분석한다. 본 연구의 결과는 스토리텔링 적용 교수학습 모형이나 방안에 대한 연구가 부족한 현재 상황에서 향후 이와 관련된 보다 다양하고 심층적인 연구를 위한 기초 자료로서 그 의의가 있을 것으로 보인다. In this study we developed a textbook for middle school mathematics, especially focusing on the 1st grade, based on storytelling, did experimental lessons using the developed textbook, and surveyed the responses of students to the lesson by three kinds of questionnaire and teacher interview. The results of this study can serve as basic data for other researches about storytelling-related education in school mathematics.