Floating zone 법에 의한 $LiNbO_3$ 단결정의 광학적 특성에 관한 연구

고정민,조현,김세훈,최종건,오근호,Ko, J.M.,Cho, H.,Kim, S.H.,Choi, J.K.,Auh, K.H. 한국결정성장학회 1995 韓國結晶成長學會誌 Vol.5 No.4

Hologen lamp를 열원으로 한 적외선 집중가열 방식의 Floating zone법을 이용하여 조화융해조성(48.6 mol% $Li_2O : 51.4 mol% LiNbO_3$)의 $LiNbO_3$ 단결정과 여기에 5 mol%의 MgO를 첨가한 $LiNbO_3$:5mod%MgO 단결정을 c-축으로 성장시켰다. 고품질의 광학용 $LiNbO_3$ 결정을 성장시키기 위해 원료봉의 최적 소결조건 및 성장 분위이게 따른 원료봉/융액 계면에 대한 고찰을 하였고, 이를 바탕으로 하여 ga s flow rate, pulling rate, 원료봉과 종자정의 회전속도 등에 대한 최적의 결정성장 조건을 확립하였다. 성장된 결정에 대해서 chemical etching을 통한 etch pattern과 ICP(Inductively Coupled Plasma) 분석을 통한 조성 변동 및 불순물 Fe 함량을 조사하였고, 5 mol% MgO의 첨가에 따른 투과율과 굴절율의 변화를 관찰하였다. 또한 $LiNbO_3$의 비선형 광학특성에 대해서는 측정한 굴절율 값을 data로 하여 비선형 광학 굴절율 $n_2$를 계산하여 다른 광학물질과 비교하였고, 이와 관련하여 비선형 광학특성에 대한 전반적인 고찰을 하였다. The c - axis oriented single crystal of $LiNbO_3$ and $LiNbO_3$ : 5mol%MgO was success-fully grown by Floating zone method using halogen lamp as a heat source. The effects of the sintering condition of the feed rod and the atmosphere gas during the crystal growth on the be havior of the feed rod/melt interface were studied for growing crystal with the high quality, and then, the optimum growth conditions were determined by studying the experimental param eters, such as gas flow rate, pulling rate, rotation speeds of the feed rod and the seed. The grown crystals were analyzed using the chemical etching to observe the tch pattern and the ICP (Inductively Coupled Plasma) to determine the composition uniformity and the impurity content of Fe. The effects of additive (5 mol % MgO) on the transmittance and refractive index was, also, analyzed. In order to compare the nonlinear optical oharacteristics of $LiNbO_3$ with those of the other optical materials, the nonlinear optical refractive index ($n_2$) was calcu l lated using the measured refractive index.

단일 기관에서 경험한 비후성 심근병증으로 발현된 영아형 폼페병

고정민,이영아,김기범,박성섭,송정한,Ko, Jung Min,Lee, Young Ah,Kim, Gi Beom,Park, Sung Sup,Song, Jung-Han 대한유전성대사질환학회 2012 대한유전성대사질환학회지 Vol.12 No.1

폼페병은 GAA 유전자의 돌연변이로 인해 acid ${\alpha}$-glucosidase (GAA) 효소가 완전 혹은 부분적으로 결핍되어 발생하는 드문 리소좀 축적질환의 하나이다. 전형적인 영아형 폼페병은 다기관을 침범하여 빠르게 진행하는 질환으로, 근긴장도 저하, 전신적인 근력 감퇴 및 비후성 심근병증이 주된 임상 양상이며 치료를 받지 않으면 보통 1-2세경에 사망에 이르게 된다. 재조합 GAA 효소 대체 요법은 이러한 영아형 폼페병 환자에서 병의 진행 경과를 늦추고 예후를 호전시키는데 효과적임이 이미 확인되었다. 본 연구에서는 단일 기관에서 경험한 비후성 심근병증으로 발현된 혈연 관계가 없는 세 명의 한국인 폼페병 환자의 임상 양상 및 유전학적 양상과 더불어 단기간의 효소 대체 요법 효과를 보고하고자 한다. 효소 대체 요법이 도입된 이래 영아형 폼페병의 자연 경과가 매우 호전된 바, 보다 조기에 진단하여 효소 대체 요법을 시작하는 것이 환자의 예후를 호전시키고 합병증을 최소화하는데 결정적일 것으로 생각된다. Pompe disease is a rare lysosomal glycogen storage disorder caused by a total or partial deficiency of the acid ${\alpha}$-glucosidase (GAA) enzyme due to the GAA gene mutations. The classic infantile form of Pompe disease is a rapidly progressive multi-organ disease with hypotonia, generalized muscle weakness, and hypertrophic cardiomyopathy, usually leading to death in the first 2 years of life. Enzyme replacement therapy with recombinant human GAA has been shown to be effective and subsequently yielded promising results. Here, we present clinical and genetic characteristics of three Korean non-classic infantile Pompe patients, and the short term efficacy of enzyme replacement therapy. Considering that enzyme replacement therapy can change the natural course of infantile Pompe disease, early diagnosis and early initiation of treatment is critical to improving patient outcomes.

조형요소인 점, 선, 면을 적용한 네일아트 디자인 연구

고정민,Ko, Jung-Min 중소기업융합학회 2020 융합정보논문지 Vol.10 No.11

본 연구는 네일아트의 시각적 표현을 위해 공간을 구성하는 조형의 기본요소인 점, 선, 면을 적용한 네일아트를 제시하여 네일아트 디자인의 창의적인 발상과 예술적인 영역의 확대에 도움이 되고자 한다. 연구방법은 전문서적과 선행연구, 인터넷 웹사이트를 통해 조형요소의 개념과 특성을 고찰하였다. 다양한 표현 기법과 함께 점, 선, 면의 특성이 네일아트에 어떻게 적용되며 시각메세지로 표현되고 있는지를 각 특성에 따라 분석하여 총 6작품을 제작하였다. 본 연구는 조형요소인 점, 선, 면이 가지는 특성을 예술적으로 접근하여 재해석함으로써 네일아트 표현에 대한 시각을 새롭게 모색하고 이를 활용하여 네일아트 디자인의 무한한 가능성을 높이는데 기여하고자 한다. This study aims to help the creative idea of nail art design and the expansion of artistic realm by presenting nail art applied with dot, line, and plane, which are the basic elements of the sculpture that make up the space for visual expression of nail art. The research method considered the concepts and characteristics of the molding elements through professional books, prior research, and Internet website. Along with various expression techniques, the characteristics of dot, line, and plane were applied to nail art and expressed as visual messages, and a total of six works were produced according to each characteristic. This research aims to find a new perspective on nail art expression by reinterpreting the characteristics of dot, line, and plane, which are formative elements, in an artistic approach, and to contribute to enhancing the infinite possibilities of nail art design by utilizing them.

발달의 퇴행을 보여 진단된 제2형 GM1 gangliosidosis 1례

고정민,조태준,채종희,Ko, Jung Min,Cho, Tae Joon,Chae, Jong-Hee 대한유전성대사질환학회 2014 대한유전성대사질환학회지 Vol.14 No.2

GM1 gangliosidosis is a rare autosomal recessively inherited metabolic disease due to deficiency of ${\beta}$-galactosidase caused by mutations in the GLB1 gene. There have been three clinical subgroups in GM1 gangliosidosis, however it is difficult to differentiate because there is considerable overlap between classical phenotypes and clinical and imaging findings among the subgroups. Here, we report a Korean girl with type 2 GM1 gangliosidosis, who showed dysostosis multiplex and progressive neurological deterioration. Developmental regression was first noted at the age of 9 months, and she was diagnosed as GM1 gangliosidosis by ${\beta}$-galactosidase enzyme analysis and GLB1 mutation analysis at the age of 16 months.

한국인 제3형 당원병 환자의 임상상 및 AGL 유전자형

고정민,이정현,김구환,유한욱,Ko, Jung Min,Lee, Jung Hyun,Kim, Gu-Hwan,Yoo, Han-Wook 대한유전성대사질환학회 2006 대한유전성대사질환학회지 Vol.6 No.1

Purpose: Glycogen storage disease type III (GSD-III), is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme is amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), which is responsible for the debranching of the glycogen molecule during catabolism. The disease has been demonstrated to show clinical and biochemical heterogeneity, reflecting the genotype-phenotype heterogeneity among different patients. In this study, we analyzed mutations of the AGL gene in three unrelated Korean GSD-III patients and discussed their clinical and laboratory implications. Methods: We studied three GSD-III patients and the clinical features were characterized. Sequence analysis of 35exons and part exon-intron boundaries of the AGLgene in patients were carried out by direct DNA sequencing method using genomic DNA isolated from patients' peripheral leukocytes. Results: The clinical features included hepatomegaly (in all patients), seizures (in patient 2), growth failure (in patients 1), hyperlipidemia (in patients 1 and 3), raised transaminases and creatinine kinase concentrations (in all patients) and mild EKG abnormalities (in patients 2). Liver transplantation was performed in patient 2due to progressive hepatic fibrosis. Administration of raw-corn-starch could maintain normoglycemia and improve the condition. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 with c.1510_1511insT (p.Y504LfsX10), and patient 3 with c.3416 T>C (p.L1139P) and c.l735+1 G>T (Y538_R578delfsX4) mutations. Except R428K mutation, 4 other mutations identified in3 patients were novel. Conclusion: GSD-III patients have variable phenotypic characteristics resembling GSD-Ia. The molecular defects in the AGL gene of Korean GSD-III patients were genetically heterogeneous.

아이소발레릭산혈증의 신생아선별검사 후 진단 및 치료 전략

고정민,이경아,Ko, Jung Min,Lee, Kyung-A 대한유전성대사질환학회 2016 대한유전성대사질환학회지 Vol.16 No.2

IVA는 상염색체 열성의 유전방식을 보이는 leucine 대사 장애 질환이자 대표적인 유기산 혈증이다. IVD 유전자의 돌연변이에 의한 isovaleryl-CoA dehydrogenase 효소의 결핍이 질환 발생의 원인이다. Isovaleryl-CoA dehydrogenase 효소가 결핍되면 isovaleryl-CoA의 대사물질이 비정상적으로 체내에 축적되어 대사성 산증 및 고암모니아혈증 등의 급성 대사성 위기와 발달지연, 성장지연, 및 경련성질환 등의 만성 합병증을 초래할 수 있기 때문에, 급성 대사성 위기 및 만성합병증의 발생을 예방하기 위해 조기 진단에 따른 적절한 치료의 도입이 중요하다. 현재 IVA은 국내에서 시행되는 탠덤 매스 스크리닝법을 이용한 신생아 대사질환 선별검사 항목에 포함되어 있으며, C5의 상승으로 의심할 수 있다. 그러나 SBCAD 결핍증 혹은 pivalic acid 유래물이 포함된 항생제를 투여한 경우에도 C5의 상승이 동반될 수 있기 때문에, 감별진단 및 확진을 위한 추가적인 생화학적, 유전학적 검사가 필수적이다. Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

한국인 스미스-렘리-오피츠 증후군 환자의 임상 양상 및 유전자형: 새로운 증례 보고 및 문헌 고찰

고정민,Ko, Jung Min 대한유전성대사질환학회 2014 대한유전성대사질환학회지 Vol.14 No.1

Smith-Lemli-Opitz syndrome (SLO) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. Here, we describe Korean siblings with SLO who were diagnosed recently, and performed a review of literature about Korean cases with SLO to date. Microcephaly and syndactyly of the second and third toes are the most common physical finding in SLOS patients. Other malformations including growth failure, cleft palate or bifid uvula, various heart malformation, genital ambiguity in males are also accompanied. Not all patients showed low levels of serum cholesterol, so DHCR7 mutation analysis can be helpful to confirmative diagnosis. Two mutations on p.R352 locus (p.R352W and p.R352Q) are commonly identified in Korean SLO patients. Although rare in Korea, SLO should be considered in the differential diagnosis of growth failure with intellectual disability, especially in patients with multiple congenital anomalies.

TWIST1 유전자의 돌연변이가 확인된 Saethre-Chotzen 증후군 2례

고정민(Jung Min Ko),양정아(Jung-Ah Yang),정선용(Seon-Yong Jeong),윤수한(Soo Han Yoon) 대한의학유전학회 2011 대한의학유전학회지 Vol.8 No.2

Saethre-Chotzen 증후군은 상염색체 우성의 유전 방식을 보이는 두개골유합증후군(craniosynostosis)의 하나로, 원인 유전자는 TWIST1로 알려져 있다. Saethre-Chotzen 증후군의 임상 증상은 두개골유합증 중에서도 특히 관상 봉합(coronal suture)이 편측 혹은 양측으로 조기에 폐쇄되는 것이 특징적이며, 이외에도 안검하수, 낮게 위치한 귀, 청력 소실, 손발가락의 기형 등 다양한 이상이 동반될 수 있다. 저자들은 양측성 관상봉합 두개골유합증과 특징적인 얼굴 모습, 다양한 동반 기형을 보인 두 명의 환자에서 TWIST1 유전자의 원인 돌연변이를 각각 확인하고 Saethre-Chotzen 증후군을 확진하였기에, 국내에서 처음으로 보고하는 바이다. 특히, TWIST1유전자 분석은 관상봉합 유합증을 보이는 환자에서 질환을 확진하고, 및 환자와 가족에 대한 적절한 유전 상담을 제공하는데 유용한 검사로 생각된다. Saethre-Chotzen syndrome is an autosomal dominant craniosynostosis syndrome, usually involving unior bilateral coronal synostosis and mild limb deformities, and is induced by loss-of-function mutations of the TWIST1 gene. Other clinical features of this syndrome include ptosis, low-set ears, hearing loss, hypertelorism, broad great toes, clinodactyly, and syndactyly. The authors of the present study report 2 children with clinical features of Saethre-Chotzen syndrome who showed mutations in the TWIST1 gene, and is the first molecular genetic confirmation of Saethre-Chotzen syndrome in Korea. The molecular genetic testing of the TWIST1 gene for patients with coronal synostoses is important to confirm the diagnosis and to provide adequate genetic counseling.

디지털융합이 영화산업에 미치는 영향에 관한 연구

고정민(Ko Jeong-Min) 한국문화산업학회 2008 문화산업연구 Vol.8 No.2

This study is to analyze the influence of digital convergence on movie industry and find the implication for its solution. Digital convergence results in three changes of value chain, industry structure and market structure. In terms of value chain, three decreases in traditional film, distribution and theater and three increases in movie file, digital transportation method and digital media can be described. And the entry of telecommunication company to movie industry in terms of industry structure and the sharp decrease of market size caused by illegal download in terms of market structure show up. In order to cope with this environmental change, the following strategies are needed. First, revenue source of new digital media should be developed and digital cinema be introduced as early as possible. Second, telecommunication companies to enter the movie industry make an effort in export of movie content. Third, the enlargement of regulation concerned with illegal download of movie through internet and the exploration of new media business model are required. This study is to analyze the influence of digital convergence on movie industry and find the implication for its solution. Digital convergence results in three changes of value chain, industry structure and market structure. In terms of value chain, three decreases in traditional film, distribution and theater and three increases in movie file, digital transportation method and digital media can be described. And the entry of telecommunication company to movie industry in terms of industry structure and the sharp decrease of market size caused by illegal download in terms of market structure show up. In order to cope with this environmental change, the following strategies are needed. First, revenue source of new digital media should be developed and digital cinema be introduced as early as possible. Second, telecommunication companies to enter the movie industry make an effort in export of movie content. Third, the enlargement of regulation concerned with illegal download of movie through internet and the exploration of new media business model are required.

국내 모바일 콘텐츠 비즈니스의 구조와 발전방향

고정민(Jeong-Min Ko),유승호(Seoung-Ho Ryu) 사이버커뮤니케이션학회 2004 사이버 커뮤니케이션 학보 Vol.- No.14

본 연구는 국내 모바일 콘텐츠 비즈니스의 구조의 분석 및 발전방향에 관한 시론적 연구이다. 국내 모바일 콘텐츠산업은 성장성, 국내 산업환경과의 적합성 등의 측면에서 타 산업에 비해 매우 중요한 의미를 가지고 있다. 그러나 최근 가입자의 포화 등 구조적인 문제를 안고 있는 모바일 콘텐츠 비즈니스가 미래 성장동력으로서 거듭나기 위해서는 시장, 공급주체 및 제도적인 면에서 새로운 발전모델의 구축이 시급하다. 따라서 본 연구는 국내 모바일 콘텐츠 비즈니스의 구조분석 및 발전방향을 제시하기 위해, 먼저 모바일 콘텐츠 산업의 성장 동인을 사회적 요인과 산업적 요인으로 구분하여 살펴보았고, 나아가서 일본과 유럽의 모바일 콘텐츠 산업을 분석함으로써 비즈니스의 전략적 시사점을 도출하였다. 결론적으로, 다음과 같이 국내 모바일 콘텐츠 비즈니스의 발전방향을 제시하였다. 첫째, 무선콘텐츠 시장자체를 확대시키기 위해 경쟁국에 앞서 킬러콘텐츠의 개발, 신모바일기기의 도입, 정액요금제, 실질적 무선망개방 등 시장을 견인할 수 있는 방안을 조기에 도입할 것을 제안하였다. 둘째, 경쟁력 있는 CP를 육성하기 위해 CP의 브랜드화 가능성을 타진하였고, 마지막으로 모바일콘텐츠 산업의 핵심 주체인 이동통신사와 CP의 상생을 위한 윈윈(win-win) 구조 방안에 대해 제시하였다. This study is the exploration that is concerned with the structure of domestic mobile contents business and its developmental direction. In domestic case, Mobile contents industry is more important than any other industry in terms of growth and conformity with Korean industry environment, symbol in Korean modem society, etc. About this, we must immediately achieve to model of developmental direction through structural analysis of the domestic mobile contents business. In order to present the structure of domestic mobile contents business and its developmental direction, we examined a growth factor of mobile industry from standpoint of both social factor and characteristics of industry. Also we analyzed mobile contents industry in Japan and Europe. In conclusion, We suggested the developmental direction of domestic mobile contents business as follows. First, by basic developmental direction, We suggested that mobile market size should be expanded by introducing driving force in advance of competitive countries such as killer contents, new mobile devices and flat fee system and opening mobile net to internet portal company and CP, etc. And to raise competitive CP, We tapped a possibility of branding CP. Finally, We suggested the device of win-win structure of both mobile network company and CP as the core of industry of mobile contents.