• Title/Summary/Keyword: Mun-myo

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Identification and Clinical Implications of Novel MYO15A Mutations in a Non-consanguineous Korean Family by Targeted Exome Sequencing

  • Chang, Mun Young;Kim, Ah Reum;Kim, Nayoung K.D.;Lee, Chung;Lee, Kyoung Yeul;Jeon, Woo-Sung;Koo, Ja-Won;Oh, Seung Ha;Park, Woong-Yang;Kim, Dongsup;Choi, Byung Yoon
    • Molecules and Cells
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    • v.38 no.9
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    • pp.781-788
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    • 2015
  • Mutations of MYO15A are generally known to cause severe to profound hearing loss throughout all frequencies. Here, we found two novel MYO15A mutations, c.3871C>T (p.L1291F) and c.5835T>G (p.Y1945X) in an affected individual carrying congenital profound sensorineural hearing loss (SNHL) through targeted resequencing of 134 known deafness genes. The variant, p.L1291F and p.Y1945X, resided in the myosin motor and IQ2 domains, respectively. The p.L1291F variant was predicted to affect the structure of the actin-binding site from three-dimensional protein modeling, thereby interfering with the correct interaction between actin and myosin. From the literature analysis, mutations in the N-terminal domain were more frequently associated with residual hearing at low frequencies than mutations in the other regions of this gene. Therefore we suggest a hypothetical genotype-phenotype correlation whereby MYO15A mutations that affect domains other than the N-terminal domain, lead to profound SNHL throughout all frequencies and mutations that affect the N-terminal domain, result in residual hearing at low frequencies. This genotype-phenotype correlation suggests that preservation of residual hearing during auditory rehabilitation like cochlear implantation should be intended for those who carry mutations in the N-terminal domain and that individuals with mutations elsewhere in MYO15A require early cochlear implantation to timely initiate speech development.

A Study on the Line of Succession to the Kim Sa-haeong of Park Ja-cheong through the Royal Tomb (왕릉건축을 통해 본 박자청(朴子靑)의 김사행(金師幸)건축 계승)

  • Kim, Bue-Dyel;Cho, Jeong-Sik
    • Journal of architectural history
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    • v.27 no.2
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    • pp.113-124
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    • 2018
  • This study investigates the relationship of two architectures 'Kim Sa-haeng' and 'Park Ja-cheong.' They were the architects whose architecture were established and well known however they were not acknowledged by the people. Because they were not revealed under the large-scale national construction in complicated circumstances at home and abroad. The results were as follows; First, Kim Sa-haeng who was an overseas architect in Yuan dynasty stood out in all areas of architecture. Particularly in making royal tombs; His works were recognized as the most beautiful royal tombs ever existed and was later on followed by the Joseon dynasty. Second, Park completed the construction of Joseon's by faithfully reflecting on the discussed process of Neo-Confucian while coming after the time of the construction of Kim Sa-haeng. Third, although Kim Sa-haeng was a vassal of the Buddhist nation in Goryeo, he built the Moon-myo, a Confucian inspired temple of Joseon. Park Ja-cheong, who continued to build Kim Sa-haeng's architecture, reproduced Kim Sa-haeng's confucian's temple of Joseon dynasty while modifying it according to the situation in Joseon dynasty. The constructions of the two architects' monuments continues unabatedly. Their architecture has continued without massive changes.

ECDIS 교육을 통해 분석한 항해사의 ECDIS 에 대한 의식

  • Jo, Myo-Jin;Hwang, Jong-O;Jo, Ik-Sun;Mun, Seong-Bae;Song, Jae-Uk
    • Proceedings of the Korean Institute of Navigation and Port Research Conference
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    • 2007.12a
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    • pp.114-116
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    • 2007
  • 최근 ECDIS 보급 및 사용이 확대됨에 따라 주요 항구에서 ECDIS 가 설치되어 있는 선박에 승선하는 항해사에게 ECDIS 자격증을 요구하고 있으므로 항해사들은 국제규정에 맞는 ECDIS 교육 과정을 이수하여야 한다. 이러한 국제적 추세에 맞추어 한국해양대학교 학교기업 마린에듀텍에서는 IMO MODEL COURSE 에 맞추어 항해사에 대한 ECDIS 교육을 수행하고 있다. 본 논문에서는 한국해양대 마린에듀텍의 교육현황 소개 및 마린에듀텍에서 ECDIS 교육을 이수한 항해사들을 대상으로 실시한 ECDIS 의식에 대한 설문 조사 결과를 소개하고자 한다.

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Alkamides from Piper longum and Piper nigrum as Inhibitors of IL-6 action

  • Lee, Seung-Woong;Kim, Myo-Sun;Park, Mi-Hye;Park, Su-Jin;Lee, Woo-Song;Chang, Jong-Sun;Rho, Mun-Chual
    • Bulletin of the Korean Chemical Society
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    • v.31 no.4
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    • pp.921-924
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    • 2010
  • Blocking of IL-6 has been postulated to be an effective therapy in the pathogenesis of several inflammatory diseases. The current study was performed to examine the potential effects of alkamides isolated from P. longum and P. nigrum on IL-6 induced Stat3 activation and identify the structure-activity relationship of these alkamides in human hepatoma cells. Among 10 alkamides isolated from P. longum and P. nigrum, compounds 6, 7 and 9 were identified as strong inhibitors of IL-6 action, which inhibit IL-6 induced Stat3-dependent luciferase activities. These inhibitory activities were positively influenced by the presence of piperidine moiety.

Expression of the Aspergillus niger var. awamori Phytase Gene in Pichia pastoris, and Comparison of Biological Properties

  • CHOI, JAE-MUN;DOO-SANG KIM;MOON-SICK YANG;HYUNG-RAK KIM;JAE-HO KIM
    • Journal of Microbiology and Biotechnology
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    • v.11 no.6
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    • pp.1066-1070
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    • 2001
  • The PhyA gene, encoding myo-inositol hexakisphosphate phosphohydrolase in Aspergillus niger var. awamori (wild-type), was cloned and sequenced. The cDNA was overexpressed by a multicopy gene expression system in Pichia pastoris KM71. Recombinant, wild-type and commercial phytase from Aspergilus ficuum NRRL 3135 (Natuphos) were purified. The PhyA gene of Aspergillus niger var awamori showed perfect homology to the phytase of Aspergillus ficcum and $97\%$ homology to A. niger var awamori (L02421). Wild-type phytase was highly glycosylated and more thermostable than the other two, while deglycosylated farms of three phytases showed identical molecular weight, 507 kDa. After heating at $80^{\circ}C$, wild-type, commercial, and recombinant phytases retained $57\%, 32%,\;and\;8\%$ of their original activities, respectively. In conclusion, glycosylation plays a key role in the thermostability of phytase and its enzymatic characterization.

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Quantitative Analysis of Genetically Modified Soybean in Processed Foods Using Real-time PCR (실시간중합효소연쇄반응을 이용한 유전자변형 콩 가공식품의 정량분석)

  • Min, Dong-Myung;Kim, Myo-Young;Jung, Soon-Il;Heo, Mun-Seok;Kim, Jin-Kuk;Kim, Hae-Yeong
    • Korean Journal of Food Science and Technology
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    • v.36 no.5
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    • pp.723-727
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    • 2004
  • Qualitative and quantitative PCR methods were performed to examine detection and quantitation of epsps inserted into genetically modified soybean (GMS) in processed foods, soy milk, tofu, and biji (soybean curd residue). Using PCR amplification to produce two (121 and 330 bp) epsps in GMS, detection limits of GMS in soy milk, tofu, and biji containing 0.01% GMS were measured. For quantitative detection, test samples containing 1, 3, and 5% GMS were measured by real-time PCR method. Results show real-time PCR method is applicable to detect GMS quantitatively in processed foods.

Improved survival rate with decreased neurodevelopmental disability in extreme immaturity (초극소저출생체중아의 생존율 향상에 따른 장기 신경발달 장애의 감소)

  • Jeon, Ga Won;Kim, Myo Jing;Kim, Sung Shin;Shim, Jae Won;Chang, Yun Sil;Park, Won Soon;Lee, Mun Hyang
    • Clinical and Experimental Pediatrics
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    • v.50 no.11
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    • pp.1067-1071
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    • 2007
  • Purpose : The aim of this study was to determine whether improved survival of extremely low birth weight infants (ELBWI) was associated with decreased neurodevelopmental disability later in life, and also to identify the factors influencing this disability. Methods : ELBWI admitted to the neonatal intensive care unit of Samsung Medical Center, survived, and followed up until the corrected age of 18 months were enrolled. They were divided into two groups according to admission time: period I (1994-1999, n=36) and period II (2000-2004, n=98). Clinical data were collected retrospectively from the medical records. Results : Survival rates increased from 60.0% to 74.7%, cerebral palsy rates decreased from 22.2% to 8.2% and catch-up growth rate increased from 25.0% to 51.0% during period I and II. Despite less gestational age and birth weight, ELBWI during period II had less periventricular leukomalacia (PVL), sepsis and bronchopulmonary dysplasia compared to period I. The highest risk factors for cerebral palsy were intraventricular hemorrhage (IVH) (${\geq}$Grade III), failure of catch-up growth and PVL. Conclusion : In summary, improved viability was associated with decreased neurodevelopmental disability in ELBWI. Improved neonatal care with resultant decrease in PVL and IVH, and better nutritional support seem to be primarily responsible for this improved outcome.

The recent essay of Bijeung - Study of III- (비증(痺證)에 대(對)한 최근(最近)의 제가학설(諸家學說) 연구(硏究) - 《비증전집(痺證專輯)》 에 대(對)한 연구(硏究) III -)

  • Yang, Tae-Hoon;Oh, Min-Suk
    • Journal of Haehwa Medicine
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    • v.9 no.1
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    • pp.513-545
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    • 2000
  • I. Introduction Bi(痺) means blocking. It can reach at the joints or muscles or whole body and make pains. Numbness and movement disorders. BiJeung can be devided into SilBi and HeoBi. In SilBi there are PungHanSeupBi, YeolBi and WanBi. In HeoBi, there are GiHyeolHeoBi, EumHeoBi and YangHeoBi. The common principle for the treatment of BiJeung is devision of the chronic stage and the acute stage. In the acute stage, BiJeung is usually cured easily but in the chronic stage, it is difficult. In the terminal stage, BiJeung can reach at the internal organs. BiJeung is one kind of symptoms making muscles, bones and jonts feel pain, numbness or edema. For example it can be gout or SLE etc. Many famous doctors studied medical science by their fathers or teachers. So the history of medical science is long. So I studied ${\ll}Bijeungjujip{\gg}$. II. Final Decision 1. BanSuMun(斑秀文) thought that BiJeung can be cured by blocking of blood stream. So he insisted that the important thing to cure BiJeung is to improve the blood stream. He usually used DangGuiSaYeokTang(當歸四逆湯), DangGuiJakYakSanHapORyeongSan, DoHong-SaMulTang(桃紅四物湯), SaMyoSanHapHeuiDongTang and HwangGiGyeJiOMulTang. 2. JangGeonBu(張健夫) focused on soothing muscles and improving blood seam. So he used many herbs like WiRyeongSeon(威靈仙), GangHwal(羌活), DokHwal(獨活), WooSeul(牛膝), etc. Especially he pasted wastes of the boiled herbs. 3. OSeongNong(吳聖農) introduced four rules to treat arthritis. So he usually used SeoGak-SanGaGam(犀角散加減), BoYanHwanOTang(補陽還五湯), ODuTang(烏頭湯), HwangGiGyeJiOMulTang. 4. GongJiSin thought disk hernia as one kind of BiJeung. And he said that Pung can hurt upper limbs and Seup can hurt lower limbs. He used to use GyeJiJakYakJiMoTang(桂枝芍藥知母湯). 5. LoJiJeong(路志正) introduced four principles to treat BiJeung. He used BangPungTang(防風湯), DaeJinGuTang) for PungBi(風痺), OPaeTang(烏貝湯) for HanBi(寒痺), YukGunJaTang(六君子湯) for SeupBi(濕痺) and SaMyoTang(四妙湯), SeonBiTang(宣痺湯), BaekHoGaGyeTang(白虎加桂湯) for YeolBi(熱痺). 6. GangChunHwa(姜春華) discussed herbs. He said SaengJiHwang(生地黃) is effective for PungSeupBi and WiRyungSun(威靈仙) is effective for the joints pain. He usually used SipJeonDaeBoTang(十全大補湯), DangGuiDaeBoTang(當歸大補湯), YoukGunJaTang(六君子湯) and YukMiJiHwanTang(六味地黃湯). 7. DongGeonHwa(董建華) said that the most important thing to treat BiJeung is how to use herbs. He usually used CheonO(川烏), MaHwang(麻黃) for HanBi, SeoGak(犀角) for YeolBi, BiHae) or JamSa(蠶沙) for SeupBi, SukJiHwang(熟地黃) or Vertebrae of Pigs for improving the function of kidney and liver, deer horn or DuChung(杜沖) for improving strength of body and HwangGi(黃?) or OGaPi(五加皮) for improving the function of heart. 8. YiSuSan(李壽山) devided BiJeung into two types(PungHanSeupBi, PungYeolSeupBi). And he used GyeJiJakYakJiMoTang(桂枝芍藥知母湯) for the treatment of gout. And he liked to use HwanGiGyeJiOMulTangHapSinGiHwan 枝五物湯合腎氣丸) for the treat ment of WanBi(頑痺). 9. AnDukHyeong(顔德馨) made YongMaJeongTongDan(龍馬定痛丹)-(MaJeonJa(馬錢子) 30g, JiJaChung 3g, JiRyong(地龍) 3g, JeonGal(全蝎) 3g, JuSa(朱砂) 0.3g) 10. JangBaekYou(張伯臾) devided BiJeung into YeolBi and HanBi. And he focused on improving blood stream. 11. JinMuO(陳茂梧) introduced anti-wind and dampness prescription(HoJangGeun(虎杖根) 15g, CheonChoGeun 15g, SangGiSaeng(桑寄生) 15g, JamSa(蠶絲) 15g, JeMaJeonJa(制馬錢子) 3g). 12. YiChongBo(李總甫) explained basic prescriptions to treat BiJeung. He used SinJeongChuBiEum(新定推痺陰) for HaengBi(行痺), SinJeongHwaBiSan(新定化痺散) for TongBi(痛痺), SinJeongGaeBiTang(新定開痺湯) for ChakBi(着痺), SinJeongCheongBiEum(新定淸痺飮) for SeupYeolBi(濕熱痺), SinRyeokTang(腎瀝湯) for PoBi(胞痺), ORyeongSan for BuBi(腑痺), OBiTang(五痺湯) for JangBi(臟痺), SinChakTang(腎着湯) for SingChakByeong(腎着病). 13. HwangJeonGeuk(黃傳克) used SaMu1SaDeungHapJe(四物四藤合制) for the treatment of a acute arthritis, PalJinHpPalDeungTang(八珍合八藤湯) or BuGyeJiHwangTangHapTaDeungTang(附桂地黃湯合四藤湯) for the chronic stage and ByeolGapJeungAekTongRakEum(鱉甲增液通絡飮) for EumHeo(陰虛) 14. GaYeo(柯與參) used HwalRakJiTongTang(活絡止痛湯) for shoulder ache, SoJongJinTongHwalRakTank(消腫鎭痛活絡湯) for YeolBi(熱痺), LiGwanJeolTang(利關節湯) for ChakBi(着痺), SinBiTang(腎痺湯) for SinBi(腎痺) and SamGyoBoSinHwan(三膠補腎丸) for back ache. 15. JangGilJin(蔣길塵) liked to use hot-character herbs and insects. And he used SeoGeunLipAnTang(舒筋立安湯) as basic prescription. 16. RyuJangGeol(留章杰) used GuMiGangHwalTang(九味羌活湯) and BangPungTang(防風湯) at the acute stage, ODuTang(烏頭湯) or GyeJiJakYakJiMoTang(桂枝芍藥知母湯) for HanBi of internal organs, YangHwaHaeEungTang(陽和解凝湯) for HanBi, DokHwalGiSaengTang(獨活寄生湯), EuiYiInTang(薏苡仁湯) for SeupBi, YukGunJaTang(六君子湯) for GiHeoBi(氣虛痺) and SeongYouTang(聖兪湯) for HyeolHeoBi(血虛痺). 17. YangYuHak(楊有鶴) liked to use SoGyeongHwalHyelTang(疏經活血湯) and he would rather use DoIn(桃仁), HongHwa(紅花), DangGui(當歸), CheonGung(川芎) than insects. 18. SaHongDo(史鴻濤) made RyuPungSeupTang(類風濕湯)-((HwangGi 200g, JinGu 20g, BangGi(防己) 15g, HongHwa(紅花) 15g, DoIn(桃仁) 15g, CheongPungDeung(靑風藤) 20g, JiRyong(地龍) 15g, GyeJi(桂枝) 15g, WoSeul(牛膝) 15g, CheonSanGap(穿山甲) 15g, BaekJi(白芷) 15g, BaekSeonPi(白鮮皮) 15g, GamCho(甘草) 15g).

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A study on the second edition of Koryo Dae-Jang-Mock-Lock (고려재조대장목록고)

  • Jeong Pil-mo
    • Journal of the Korean Society for Library and Information Science
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    • v.17
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    • pp.11-47
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    • 1989
  • This study intends to examine the background and the procedure of the carving of the tablets of the second edition of Dae-Jang-Mock­Lock(재조대장목록). the time and the route of the moving of the tablets. into Haein-sa, and the contents and the system of it. This study is mainly based on the second edition of Dae-Jang-Mock-Lock. But the other closely related materials such as restored first. edition of the Dae- Jang-Mock-Lock, Koryo Sin-Jo-Dae-Jang-Byeol-Lock (고려신조대장교정별록). Kae-Won-Seok-Kyo-Lock (개원석교록). Sok-Kae­Won-Seok-Kyo-Lock (속개원석교록). Jeong-Won-Sin-Jeong-Seok-Kyo­Lock(정원신정석교록), Sok-Jeong-Won-Seok-Kyo-Lock(속정원석교록), Dea-Jung-Sang-Bu-Beob-Bo-Lock(대중상부법보록), and Kyeong-Woo-Sin-Su-Beob-Bo-Lock(경우신수법보록), are also analysed and closely examined. The results of this study can be summarized as follows: 1. The second edition of Tripitaka Koreana(고려대장경) was carved for the purpose of defending the country from Mongolia with the power of Buddhism, after the tablets of the first edition in Buin-sa(부이사) was destroyed by fire. 2. In 1236. Dae-Jang-Do-Gam(대장도감) was established, and the preparation for the recarving of the tablets such as comparison between the content, of the first edition of Tripitalk Koreana, Gal-Bo-Chik-Pan-Dae­Jang-Kyeong and Kitan Dae- Jang-Kyeong, transcription of the original copy and the preparation of the wood, etc. was started. 3. In 1237 after the announcement of Dae-Jang-Gyeong-Gak-Pan-Gun­Sin-Gi-Go-Mun(대장경핵판군신석고문), the carving was started on a full scale. And seven years later (1243), Bun-Sa-Dae-Jang-Do-Gam(분사대장도감) was established in the area of the South to expand and hasten the work. And a large number of the tablets were carved in there. 4. It took 16 years to carve the main text and the supplements of the second edition of Tripitaka Koreana, the main text being carved from 1237 to 1248 and the supplement from 1244 to 1251. 5. It can be supposed that the tablets of the second edition of Tripitaka Koreana, stored in Seon-Won-Sa(선원사), Kang-Wha(강화), for about 140 years, was moved to Ji-Cheon-Sa(지천사), Yong-San(용산), and to Hae-In-Sa(해인사) again, through the west and the south sea and Jang-Gyeong-Po(장경포), Go-Ryeong(고령), in the autumn of the same year. 6. The second edition of Tripitaka Koreana was carved mainly based on the first edition, comparing with Gae-Bo-Chik-Pan-Dae-Jang-Kyeong(개보판대장경) and Kitan Dae-Jang-Kyeong(계단대장경). And the second edition of Dae-Jang-Mock-Lock also compiled mainly based on the first edition with the reference to Kae-Won-Seok-Kyo-Lock and Sok-Jeong-Won-Seok-Kyo-Lock. 7. Comparing with the first edition of Dae-Jang-Mock-Lock, in the second edition 7 items of 9 volumes of Kitan text such as Weol-Deung­Sam-Mae-Gyeong-Ron(월증삼매경론) are added and 3 items of 60 volumes such as Dae-Jong-Ji-Hyeon-Mun-Ron(대종지현문논) are substituted into others from Cheon chest(천함) to Kaeng chest(경함), and 92 items of 601 volumes such as Beob-Won-Ju-Rim-Jeon(법원주임전) are added after Kaeng chest. And 4 items of 50 volumes such as Yuk-Ja-Sin-Ju-Wang-Kyeong(육자신주왕경) are ommitted in the second edition. 8. Comparing with Kae-Won-Seok-Kyo-Lock, Cheon chest to Young chest (영함) of the second edition is compiled according to Ib-Jang-Lock(입장록) of Kae-Won-Seok-Kyo-Lock. But 15 items of 43 vol­umes such as Bul-Seol-Ban-Ju-Sam-Mae-Kyeong(불설반주삼매경) are ;added and 7 items of 35 volumes such as Dae-Bang-Deung-Dae-Jib-Il­Jang-Kyeong(대방등대집일장경) are ommitted. 9. Comparing with Sok-Jeong-Won-Seok-Kyo-Lock, 3 items of the 47 volumes (or 49 volumes) are ommitted and 4 items of 96 volumes are ;added in Caek chest(책함) to Mil chest(밀함) of the second edition. But the items are arranged in the same order. 10. Comparing with Dae- Jung-Sang-Bo-Beob-Bo-Lock, the arrangement of the second edition is entirely different from it. But 170 items of 329 volumes are also included in Doo chest(두함) to Kyeong chest(경함) of the second edition, and 53 items of 125 volumes in Jun chest(존함) to Jeong chest(정함). And 10 items of 108 volumes in the last part of Dae-Jung-Sang-Bo-Beob-Bo-Lock are ommitted and 3 items of 131 volumes such as Beob-Won-Ju-Rim-Jeon(법원주임전) are added in the second edition. 11. Comparing with Kyeong-Woo-Sin-Su-Beob-Bo-Lock, all of the items (21 items of 161 volumes) are included in the second edition without ;any classificatory system. And 22 items of 172 volumes in the Seong­Hyeon-Jib-Jeon(성현집전) part such as Myo-Gak-Bi-Cheon(묘각비전) are ommitted. 12. The last part of the second edition, Joo chest(주함) to Dong chest (동함), includes 14 items of 237 volumes. But these items cannot be found in any other former Buddhist catalog. So it might be supposed as the Kitan texts. 13. Besides including almost all items in Kae-Won-Seok-Kyo-Lock and all items in Sok-Jeong-Won-Seok-Kyo-Lock, Dae-Jung-Sang-Bo­Beob-Bo-Lock, and Kyeong-Woo-Sin-Su-Beob-Bo-Lock, the second edition of Dae-Jang-Mock-Lock includes more items, at least 20 items of about 300 volumes of Kitan Tripitaka and 15 items of 43 volumes of traditional Korean Tripitake that cannot be found any others. Therefore, Tripitaka Koreana can be said as a comprehensive Tripitaka covering all items of Tripitakas translated in Chinese character.

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