• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.99-106
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• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• Korean Journal of Heritage: History & Science
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• v.46 no.1
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• pp.268-289
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• 2013

The Dinosaur tracksite at Jeori, Geumseongmyeon, Euiseonggun, Gyeongsangbukdo, Korea (National Monument No. 373) has been studied in the aspects of location, stratigraphy, sedimentology, fossil occurrence, unique geological records, literature, significance in natural history, preservation, and management. On the basis of these features, the Jeori tracksite has been assessed semiquantitavely. The Jeori tracksite occurs in the Sagok Formation (Albian) of the Euiseong sub-basin, and over 300 footprints forming 12 sauropod trackways, 10 ornithopod trackways, and 1 theropod trackways are preserved in this tracksite. The track-bearing deposits consist of tabular-bedded medium- to fine-grained arkose with mudstone drape, interlaminated fine-grained sandstone to siltstone and mudstone, and shaly mudstone. The dinosaur tracks are preserved in the interlaminated fine-grained sandstone to siltstone and mudstone, and most of them are observed as underprints. The track-bearing deposits are interpreted as sheetflood deposits on the floodplain under a seasonal paleoclimatic condition with alternating of wetting and drying periods. Multiple tension fractures with NE strike were formed in the track-bearing bed, which resulted in that tracks seem to occur in several horizons. The significance in natural history of the tracksite can be summarized as follows: 1) the historical implication of the Jeori tracksite as the firstly designated National Monument of dinosaur fossil sites, 2) the high density of the occurrence of diverse footprints (over 300) within small area (about $1,600m^2$), and 3) the significance of the tension fractures associated with the track-bearing bed as geoeducational records for the understanding the development of fault. In order to share the value of the Jeori tracksite in the aspect of natural history with the community and public, the interpretive panel should be modified to include figures explaining paleoenvironment and tension fault development. In addition it is recommended that a brochure be published briefly explaining the tracksite and to educate the residents about the natural and social significance of the tracksite. For the safety of visitors it would be desirable for the road in front of the tracksite to be moved at least 10 m southward, which could mitigate the shaking of the track bed caused by traffic.

• Journal of The Korean Association For Science Education
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• v.40 no.6
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• pp.595-609
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• 2020

The purpose of this study is to analyze the experiences of teachers who participated in the development of online science class videos in the context of covid-19, their perception of online science class, and the characteristics of the online science class content developed by teachers. A survey and interviews were conducted with ten elementary school teachers who made online science class videos themselves. Also the characteristics of the online science class were investigated by analyzing the online science class video produced by the participants. As a result, participants in the study recognized the lack of production time, difficulty in filming and editing, concerns over misconceptions, the problem of solving copyrights for existing materials, and the burden of external disclosure. Although it was a teacher who had experience producing online science class video contents, no research participants actively answered the merits of online science class. On the other hand, the study participants cited that the shortcomings of online science classes were that students had fewer opportunities for inquiry and lack of communication or interaction. In particular, these shortcomings were thought to have a great influence on the quality of online science classes, especially in making inquiry classes difficult. Some teachers took a negative view that online science classes could not completely replace face-to-face classes. However, if multiple teachers are presented with supplementary teaching activities that complement the content-based online teaching method, the method of combining online science classes and face-to-face classes is not. Through the analysis of the contents of the online science class, the introduction and arrangement steps of the online science class were similar to the process of the face-to-face science class, but the inquiry step and the conceptual explanation step showed a big difference from the face-to-face science class.

• Journal of The Korean Association For Science Education
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• v.42 no.4
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• pp.417-428
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• 2022

This study analyzed the frequency and level of scientific practices presented in secondary science textbooks. A total of 1,378 student activities presented in 14 middle school science textbooks and 5 high school integrated science textbooks were analyzed, using the definition and level of scientific practice suggested in the NGSS. Findings show that most student activities focus on three practices. Compared to the textbooks for the previous science curriculum, the practice of 'obtaining, evaluating, and communicating information' was more emphasized, reflecting societal changes due to ICT development. However, the practice of 'asking a question', which can be an important element of student-led science learning, was still rarely found in textbooks, and 'developing and using models', 'using math and computational thinking' and 'arguing based on evidence' were not addressed much. The practices were mostly elementary school level except for the practice of 'constructing explanations'. Such repeated exposures to a few and low level of practices mean that many future citizens would be led to a naïve understanding of science. The findings imply that it is necessary to emphasize various practices tailored to the level of students. In the upcoming revision of the science curriculum, it is necessary to provide the definition of practices that are not currently specified and the expected level of each practice so that the curriculum can provide sufficient guidance for textbook writing. These efforts should be supported by benchmarking of overseas science curriculum and research that explore students' ability and teachers' understanding of scientific practices.

• Asia-Pacific Journal of Business Venturing and Entrepreneurship
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• v.18 no.5
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• pp.91-107
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• 2023

Lean startup is a concept that combines the words "lean," meaning an efficient way of running a business, and "startup," meaning a new business. It is often cited as a strategy for minimizing failure in early-stage businesses, especially in software-based startups. By scrutinizing the case of a startup L, this study suggests that lean startup methodology(LSM) can be useful for hardware and manufacturing companies and identifies ways for early startups to successfully implement LSM. To this end, the study explained the core of LSM including the concepts of hypothesis-driven approach, BML feedback loop, minimum viable product(MVP), and pivot. Five criteria to evaluate the successful implementation of LSM were derived from the core concepts and applied to evaluate the case of startup L . The early startup L pivoted its main business model from defecation alert system for patients with limited mobility to one for infants or toddlers, and finally to a smart bottle for infants. In developing the former two products, analyzed from LSM's perspective, company L neither established a specific customer value proposition for its startup idea and nor verified it through MVP experiment, thus failed to create a BML feedback loop. However, through two rounds of pivots, startup L discovered new target customers and customer needs, and was able to establish a successful business model by repeatedly experimenting with MVPs with minimal effort and time. In other words, Company L's case shows that it is essential to go through the customer-market validation stage at the beginning of the business, and that it should be done through an MVP method that does not waste the startup's time and resources. It also shows that it is necessary to abandon and pivot a product or service that customers do not want, even if it is technically superior and functionally complete. Lastly, the study proves that the lean startup methodology is not limited to the software industry, but can also be applied to technology-based hardware industry. The findings of this study can be used as guidelines and methodologies for early-stage companies to minimize failures and to accelerate the process of establishing a business model, scaling up, and going global.