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Background X-linked spondyloepiphyseal dysplasia tarda (SEDT-XL) is a skeletal disorder characterized by defective structures of vertebral bodies and/or of epiphyses of the long bones, resulting in moderately short stature and early joint degeneration. TRAPPC2 gene, which is important for collagen secretion, has been reported as causative for SEDT-XL. Case presentation Here, we report two variants of TRAPPC2 gene of SEDT-XL patients, a missense variant of start codon, c.1A > T, and a deletion variant, c.40delG. To understand molecular consequence of the variants, we establish an in vitro gene expression assay system and demonstrate that both mutated genes are transcribed, but are not properly translated, indicative of the pathogenic nature of those TRAPPC2 variants. Conclusions In the current study, we provide additional experimental data showing that loss-of-function TRAPPC2 variants are probably causative for SEDT-XL phenotype. These findings further contribute to the understanding the clinical picture related to TRAPPC2 gene. ; TJC is supported by National Research Foundation (NRF) funded by the Ministry of Science, ICT & Future Planning of the Korean government (NRF2014M3C9A2064684: Genome Technology to Business Translation Program), which has been used for the patient recruitment and care, and determining genetic variants. YK is supported by NRF funded by the Ministry of Science, ICT & Future Planning of the Korean government (NRF-2014M3C9A2064688: Genome Technology to Business Translation Program and RF2016R1A5A1011974), which have been used for validation of the pathogenicity of identified variants and in vitro functional studies. All the decisions regarding to the current studies are made by authors, not by funders. The funders are not involved in the study design, data collection and analysis, performing experiments and in writing the manuscript.
학위논문 (석사)-- 서울대학교 대학원 : 행정학과, 2012. 2. 김상헌. ; 중앙정부는 지방정부의 부족한 재원을 지방교부세, 국고보조금과 같은 지방재정조정제도를 통해 보전하고 있다. 우리나라의 경우 지방 재정조정제도의 하나인 지방교부세에 보통교부세와 특별교부세가 있 다. 특별교부세는 보통교부세의 경직적 산정에 따른 배분의 한계를 보완하여 각 지방재정력의 격차를 고려하여 보전한다는 방침으로 행 정안전부에서 운영하고 있다. 그러나 특별교부세는 배분의 기준과 결 정이 모호하고 불투명하여 그 결정이 정치적으로 이루어진다는 문제 점을 지적하는 연구가 있다. 선행연구로 이루어진 특별교부세 배분에 관한 실증분석에서는 배분의 결정에 영향을 미친다고 상정한 정치적 행위자를 국회 행정안전위원회 소속, 지역구 국회의원 수 등 의회의 영역으로만 한정하였다. 본 논문은 정치적 요인에 의회의 행위자뿐만 아니라 정부의 행위자 를 포함시켜 분석하였다. 본 논문은 전국 234개 시‧군‧구의 2003~2006년도 4개년도의 특별교부세 자료를 이용하여 특별교부세 배분의 결정에 영향을 미치는 요인들에 대해 정부 정무직 공직자를 포함하여 실증분석을 시도하였다. 그 결과 정부 정무직 공직자로 상 정한 변수가 배분의 결정에 영향을 미친다고 파악되었고, 정부 정무 직 공직자 중에서도 국무총리와 국무조정실‧차장이 다른 정무직 공직 자에 비해 더 큰 영향을 미친다고 파악되었다. 특별교부세 배분에 정무직 공직자가 자기 출생지에 더 많이 배분하 려 결정 과정에 영향을 미치는 것뿐만 아니라 정무직 공직자가 임기 후 선출직에 출마할 경우 특별교부세 배분의 결정에 더 큰 영향을 미 친다는 것을 보았다. 이로써 특별교부세 배분의 결정에 정부의 정무 직 공직자가 영향을 미친다는 것을 확인할 수 있었다. ; The national government makes up for any shortfall in finance of the local governments by the local finance coordination system, which includes local revenue sharings and national subsidies. The local revenue sharing, as a kind of the local finance coordination system, consists of an ordinary revenue sharing and a special revenue sharing. The latter is introduced to remedy the shortcomings of the former's allocation system caused by the rigidity of its calculation, and is currently operated by the Ministry of Public Administration and Security. There are some literature criticizing the problem that the SRS allocation is decided politically due to the fact that the principles for its allocation are equivocal and the decisions are arbitrary and not transparent. In previous studies analyzing the SRS allocation empirically, the scope of the political actors who are assumed to exert influences on the allocation decision is limited to the legislative area, such as the members of the National Assembly Public Administration and Security Committee and the number of lawmakers in each electoral district. This study presents a further analysis with a new political factor, the government actors, in addition to the existing legislative actors. Specifically, using the SRS data for the period of 2003 ~ 2006 for the entire 234 cities- counties-districts in the country, the present study attempts to analyze the factors, which affect SRS allocation decisions, including the new variable, the government's political masters. The results demonstrate that the new variable indeed affects the allocation decisions and that, among the political masters, prime ministers and directors generals and deputy directors in the Prime Ministers Office are shown to exert greater influences than others. Moreover, it is also shown that they exercise their power in the SRS allocation decision making process to provide more sharings to their places of birth, and in particular, their intervention is even greater when they decide to run for an elective office from their places of birth after the termination of their government offices. As such, this study verifies that the government's political masters exert influences on the SRS allocation decision. ; Master
