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Characterization of a Mucolipidosis Type II Mouse Model and Therapeutic Implication of Lysosomal Enzyme Enriched Fraction Derived from Placenta (뮤코지방증 2형 마우스 모델의 특징과 태반에서 추출한 리소좀 효소 투여의 결과)

  • Cho, Sung Yoon;Kim, Ki-Yong;Kim, Su Jin;Sohn, Young Bae;Maeng, Se Hyun;Kim, Chi Hwa;Ko, Ah-Ra;Song, Junghan;Yeau, Sung-Hee;Kim, Kyung-Hyo;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.5-13
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    • 2012
  • I-cell disease (mucolipidosis type II; MIM 252500) and pseudo-Hurler polydystrophy (mucolipidosis type III; MIM 252600) are disorders caused by abnormal lysosomal transport in cells. The presence of numerous inclusion bodies in the cytoplasm of fibroblasts, a lack of mucopolysacchariduria, increased lysosomal enzyme activity in serum, and decreased GlcNAc-phosphotransferase activity are hallmark. Here, we attempted to investigate phenotypical and biochemical characteristics of the knockoutmouse of GlcNAc-phosphotransferase ${\alpha}/{\beta}$ subunits; in addition, we also attempted to determine whether the lysosome enriched fraction derived from placenta can be beneficial to phenotype and biochemistry of the knockout mouse.We found that the knockout mouse failed to thrive and had low bone density, as is the case in human. In addition, skin fibroblasts from the animal had the same biochemical characteristics, including increased lysosomal enzyme activity in the culture media, in contrast to the relatively low enzyme activity within the cells. Intravenous injection of the lysosome rich fraction derived from placenta into the tail vein of the animal resulted in a gain of weight, while saline injected animals didn't.In conclusion, our study demonstrated the phenotypical and biochemical similarities of the knockout mouse to a mucolipidosis type II patient and showed the therapeutic potential of the lysosome enriched fraction. We admit that a larger scale animal study will be needed; however, the disease model and the therapeutic potential of the lysosome enriched fraction will highlight the hope for a novel treatment approach to mucopolipidosis type II, for which no therapeutic modality is available.

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Edible Films from Protein Concentrates of Rice Wine Meal (주박 단백질 농축물로부터 가식성필름의 제조)

  • Cho, Seung-Yong;Park, Jang-Woo;Rhee, Chul
    • Korean Journal of Food Science and Technology
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    • v.30 no.5
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    • pp.1097-1106
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    • 1998
  • Biodegradable edible films were prepared from rice protein concentrates (RPC) made from rice wine meal by alkaline extraction and isoelectric precipitation. The effect of film forming solution pH and plasticizers were studied, and cross-linkers were added to improve mechanical properties and water vapor permeabilities (WVP) of films. Films could be formed within pH $8{\sim}11$ with tensile strength (TS) of 4.3{\sim}5.7\;MPa$. Films produced under pH 11 had the highest TS (5.7 MPa) and the lowest WVP $(0.44\;ng{\cdot}m/m^2{\cdot}s{\cdot}Pa)$. Added glycerol, polyethylene glycol 200 (PEG) and its mixture (GLY:PEG=50:50) as plasticizers also affected the mechanical properties and WVP of films. TS and elongation at break (E) of films at various plasticizer levels were $5.5{\sim}1.0\;MPa$ and $3.6{\sim}24.3%$, respectively. At the same plasticizer concentration, the highest TS was observed when glycerol was used whereas the highest E was measured when mixture was used as plasticizer. WVPs of films with thickness of $60\;{\mu}m$ were $0.39{\sim}0.54\;ng{\cdot}m/m^2{\cdot}s{\cdot}Pa$. WVP of films decreased as the ratio of glycerol/PEG 200 was decreased, and WVP increased as the total amount of plasticizer added to the films increased. Film strength was improved by the addition of small amount of sodium hydrogen sulfate, succinic anhydride, ascorbic acid and citric acid, whereas TS of films containing $0.5{\sim}2.0%$ of NaCl and $CaCl_2$ were lower than those without the salts. The highest TS (6.3 MPa) was achieved with films containing 0.1% of succinic anhydride.

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Studies on Standard Physical Growth and Development by Age and Body Height in Korean Youth (한국인(韓國人)의 연령(年齡) 및 신장별(身長別)에 따른 표준체격치(標準體格値)에 관(關)한 연구(硏究) -7세(歲)부터 20세(歲)까지의 남여(男女)를 중심(中心)으로-)

  • Ahn, Kwang-Tai;Park, Soon-Young;Park, Yang-Won
    • Journal of Preventive Medicine and Public Health
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    • v.17 no.1
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    • pp.145-172
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    • 1984
  • In order to assess the physical growth pattern of Korean Youth, the authors measured the body height, body weight, chestgirth and sitting height of 40967 persons (24832 males and 16135 females) from primary schools, middle and high schools and colleges of metropolitan (urban) and rural areas, and calculated the mene, standard deviation, standard error and coefficient variance of the body weight chestgirth, sitting height and various pertinent index by body height to demonstrate the standard physical growth and development by body height of sex and age. The following conclusions were obtained. 1. Physical growth and development: Rapid growth of physical growth in terms of body height has been observed among males in the age $7{\sim}15$ and among female $7{\sim}13$. Growth in terms of body height turned out to be slower among students of higher age by both sexes. The age of cross over between to sexes is between 10 to 13 years where upon girls out grows boys. Maximum annual growth were upon girls out grows boys. Maximum annual growth were both of 6.16cm from 8 to 9 years old and 12 to 13 years old for boys and 7.2cm from 8 to 9 and 6.1cm from 9 to 10 for girls. This indicates that girls enter a rapidly growing stage 2 years earlier than boys. Meanwhile, prominent improvement in body height of national students over period of ten year was noticed. 2. The distribution status of body height by age: The distribution status of body height by age were as follows; 7 year of age: boys-30cm range of body height from 104.0cm to 133.9cm, girls-27cm from 104.0cm to 130.9cm 8 year of age: boys-30cm from 116.0 to 145.9cm girls-33cm from 113.0 to 145.9cm 9 year of age: boys-30cm from 116.0 to 145.9cm girls-33cm from 113.0 to 145.9cm 10 year of age: boys-39cm from 116.0 to 154.9cm girls-39cm from 119.0 to 157.9cm 11 year of age: boys-45cm from 119.0 to 163.9cm girls-39cm from 122.0 to 160.9cm 12 year of age: boys-45cm from 125.0 to 169.9cm girls-42cm from 125.0 to 166.9cm 13 year of age: boys-45cm from 128.0 to 172.9cm girls-42cm from 128.0 to 169.9cm 14 year of age: boys-48cm from 131.0 to 178.9cm girls-36cm from 134.0 to 169.9cm 15 year of age: boys-42cm from 137.0 to 181.9cm girls-33cm from 137.0 to 169.9cm 16 year of age: boys-39cm from 146.0 to 184.9cm girls-30cm from 143.0 to 172.9cm 17 year of age: boys-39cm from 146.0 to 184.9cm girls-27cm from 143.0 to 169.9cm 18 year of age: boys-36cm from 152.0 to 187.9cm girls-27cm from 146.0 to 172.9cm 19 year of age: boys-30cm from 155.0 to 184.9cm girls-24cm from 146.0 to 169.9cm 20 year of age: boys-24cm from 158.0 to 181.9cm girls-l8cm from 149.0 to 166.9cm 3. Standard values of body weight, chest-girth and sitting height by body height of age were found all age groups from 7 to 20 years old and listed in tables from3-a to 16-a. 4. Standard values of relative body weight, relative chestgirth and relative sitting height by body height of age were found all age groups from 7 to 20 years old and listed in tables from 3-b to 16-b. 5. Standard values of physical and nutritional indices (Rohrer index, Kaup index, Vervaeck index and Pelidisi index) by body height of age were found all age groups from 7 to 20 years old and listed in tables from 3-c to 16-c.

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Health Assessment of Shift Workers in a Automobile Manufacturing Plant (자동차공장 교대작업 근로자들의 건강상태평가)

  • Lee, Jung-Jeung;Chung, Jong-Hak
    • Journal of Preventive Medicine and Public Health
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    • v.28 no.1 s.49
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    • pp.103-121
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    • 1995
  • An investigation on psychophysiological health and social well-being of shift workers been carried out on workers of a automobile manufacturing plant in Ulsan, for 1 month from April, 1993. This cross-sectional survey compared shift workers(n=544) with day workers(n=115). Each subject completed a questionnaire about his personal habit, background, shift schedule, sleep and eating patterns, subjective digestive symptom and psychological well-being and distress using the General Well-Being Schedule(GWB) by self administrated questionnaire that was developed for the U.S. Health and Nutrition Examination Surveys (HANES I). Chi-square analysis was used for discontinuous data and the t-test was used for continous data to determine whether differences noted between the two groups. In terms of sleep quality, a greater percentage of shift workers frequently had trouble getting back to sleep once awakend(p<0.01), and a greater percentage of shift workers awakend tired or sleepy frequently more often than day workers(p<0.05). In rating the quality of their sleep, more shift workers rated their sleep fair to poor than day workers, and greater percentage of shift workers felt tired or sleepy at work two or more times per week(p<0.01) and a much higher percentage of shift workers felt tired or sleepy after work every days(p<0.01). In terms of sleep patterns, a much higher percentage of day workers reported uninterrupted sleep per 24hours than shift workers. The shift workers reported different eating patterns from day workers but there were no statistically significant and rate of their appetite. Thirteen percent of day workers reported the best appetite but only 6.6% of shift workers had the best appetite. The gastric complaints is more frequent in shift workers than day workers(p<0.01). Among subscores in General Well-Being Schedule, anxiety, depression, positive well-being and vitality subscale of shift workers were lower than those of day workers(p<0.05) and general health and self control subscale of shift workers were lower than .those of day workers but there were no statistical significant difference. Based on these study result, it could be concluded that the shift work has significant effects on some psychophysiological conditions of the workers.

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Seed Set in Close Pollination and Backcross of Interspecific F1 of Lilium spp. (백합 근연수분 및 종간 교잡종 F1 여교잡시 종자형성)

  • Lee, Ji-Yong;Lim, Yong-Pyo
    • Journal of Plant Biotechnology
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    • v.30 no.4
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    • pp.353-357
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    • 2003
  • We studies seed set in the interspecific F1 /backcross hybrids of Lilium species. In the interspecfic hybrid of L. longiflorum cv. Gelria with L.${\times}$ fomolongi cv. Raizan,93% fruit set was obtained by stigmatic pollination in comparison to 53% from cut-style pollination. Accordingly, the number of seed set resulting from stigmatic and cut-style pollination was 147 and 53, respectively. Pollination o( both stigmatic and cut-style pollination resulted in 47% fruit set in the hybrid of L. longiflorum cv. Lorina with L.${\times}$ fomolongi cv. Raizan. However, stigmatic pollination formed 413 seeds, whereas only 24 seeds were obtained by cut-style pollination in this cross. The hybrid of L.${\times}$ fomolongi cv. Raizan with L. longiflorum cv. Come set 40% fruit with a total of 43 seeds by stigmatic pollination. However, no fruit set was observed in cut-style pollination in this hybrid. Backcrossing the F1 hybrid by cut-style pollination of L. longiflorum cv. Lorina ${\times}$ Asiatic hybrid cv. Chicago with the latter parent led to 53% fruit set, and 109 embryos were obtained. Likewise, backcrossing following cut-style pollination of L. longiflorum cv. Lorina ${\times}$ Asiatic hybrid cv. Corsia with the latter parent formed 67% fruits and 107 embryos. However, in the remaining interspecific hybrids, cut-style pollination set no fruit.

Effects of Post Individualities on Treatability of Small Diameter Japanese Larch (Larix Leptolepis) with ACQ and CCA (낙엽송 원주상 소경목의 원목성상과 방부 처리성)

  • Kim, Yeong-Suk;Hong, Soon-Il;Yun, Jeong-Hee
    • Journal of the Korean Wood Science and Technology
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    • v.34 no.1
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    • pp.52-60
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    • 2006
  • This study investigated how wood properties (i.e., annual rings, sapwood, heartwood, and cracks) might affect preservative treatment in Japanese larch (Larix leptolepis) round-wood product. We specially focused on small-diameter (~10 cm diameter) wood that is commercially sold in market. Among 100 wood samples, the groups of sample with 13~16, 17~20 annual rings represented 33 and 27 in each frequency, while 2~3 and 4~5 mm annual ring width accounted for 72 and 68 in frequency. More than a half (54%) of wood samples contained a mix of heartwood and sapwood in its surface. The rest (46%) had only heartwood exposed in the wood surface. A wide range of checks were showed in the wood samples, but the highest frequency was observed in samples with 1~6 surface (1~14 mm in size) checks and 1~4 end-grain (8~14 mm in size) checks in each round-wood sample. Pressure treatment resulted in a wide range of penetration of ACQ (Alkaline Copper Quat) into the wood, showing $4.3{\pm}4.19mm$ penetration in the wood samples contained a mix of heartwood and sapwood in its surface. However preservative treatment was much less effective for the heartwood only wood samples, ranging average 1.3 mm with ACQ and 1.1 mm with CCA (Chromated Copper Arsenate). These penetration results shown in heartwood samples did not meet the penetration standard that is required for H3 by the Korean Forest Service in relation to wood preservation treatment. These low penetration results were not significantly improved even if we incised wood samples to improve treatment effect, showing only small increase of 0.7 mm with ACQ and 0.6 mm with CCA. When preservative treatment was tested with heartwood, penetration of preservatives decrease with increase of annual rings per a cross-section area (r=0.5345). We also found that the length and number of check had no effect on preservative treatment, showing r=0.1301 and r=0.1802, respectively.

Performance Measurements of Positron Emission Tomography: An Investigation Using General Electric $Advance^{TM}$ (양전자방출단층촬영기의 표준 성능평가 방법: GE $Advance^{TM}$에 적용한 예)

  • Lee, J.R.;Choi, Y.;Choe, Y.S.;Lee, K.H.;Kim, S.E.;Shin, S.A.;Kim, B.T.
    • The Korean Journal of Nuclear Medicine
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    • v.30 no.4
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    • pp.548-559
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    • 1996
  • A series of performance measurements of positron emission tomography (PET) were performed following the recommendations of the Computer and Instrumentation Council of the Society of Nuclear Medicine and the National Electrical Manufacturers Association. We investigated the performance of the General Electric $Advance^{TM}$ PET. The measurements include the basic intrinsic tests of spatial resolution, scatter fraction, sensitivity, and count rate losses and randoms. They also include the tests of the accuracy of corrections: count rate linearity correction, uniformity correction, scatter correction and attenuation correction. GE $Advance^{TM}$ PET has bismuth germanate oxide crystals (4.0mm transaxial ${\times}$ 8.1mm axial ${\times}$ 30.0mm radial) in 18 rings, which form 35 imaging planes spaced by 4.25mm. The system has retractable tungsten septa 1mm thick and 12cm long. Transaxial resolution was 4.92mm FWHM in 2D and 5.14mm FWHM in 3D at the center. Average axial resolution in 2D decreased from 3.91mm FWHM at the center to 6.49mm FWHM at R=20cm. Average scatter fraction of direct and cross slices was 9.57%. Dead-time losses of 50% corresponded to a radioactivity concentration of $4.86{\mu}Ci/cc$ and a true count rate of 519 kcps in 2D. The accuracy of count rate linearity correction was 1.84% at the activity of $4.50{\mu}Ci/cc$. Non-uniformity was 2.06% in 2D and 2.93% in 3D. Remnant errors after scatter correction were 0.55% in 2D and 4.12% in 3D. The errors of attenuation correction were 6.21% (air), 0.20% (water), -6.32% (teflon) in 2D and 5.00% (air), 6.94% (water), 3.01% (teflon) in 3D. The results indicate the performance of GE $Advance^{TM}$ PET scanner to be well suited for clinical and research applications.

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Direct Action of Relaxin on the Pig Mammary Glands to Promote Development of the Alveoli and Remodeling of the Extracellular Matrix (Relaxin Hormone의 직접젇인 작용에 의한 돼지 유선상피세포의 발달과 세포외간 기질의 분해개조 촉진)

  • Min, G.S.
    • Journal of Animal Science and Technology
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    • v.46 no.4
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    • pp.585-592
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    • 2004
  • Our previous studies demonstrated that relaxin in concert with estrogen promotes development of the mammary parenchyma during the last third of gestation in gilts, and the specific relaxin-binding sites were present in the mammary gland. This study was conducted to determine if relaxin-binding sites in the mammary gland were functional relaxin receptors. Three cycling cross-bred gilts were bilaterally ovariectomized on day 0 of the experiment. Beginning on day 15 and continuing through day 29 post-surgery, the gilt received an im. injection of estradiol benzoate at 12-hr intervals. Beginning on day 22 post-surgery, highly purified porcine relaxin was administered(lug/hr) into the left fourth mammary gland from the anterior end via miniature osmotic pump. Physiological saline was administered to the right fourth mammary gland. The gilt was sacrificed on day 29 post-surgery and histological characteristics of the mammary parenchyma were examined. The mammary glands treated locally with saline showed little, if any, lobulo-alveolar development, whereas the mammary glands treated with relaxin showed not only marked lobulo-alveolar development but also prominent secretions in the alveoli. The saline-treated glands were characterized by relatively dense and highly organized collagen fiber bundles. Whereas, in the relaxin-treated mammary glands, collagen fiber bundles were dispersed and loosely organized. In conclusion, relaxin-binding sites in the mammary gland are functional relaxin receptors and relaxin acts directly on the pig mammary gland to promote development of the alveoli and remodeling of the extracellular matrix.

Studies on the N-Compounds during Chung-Kook-Jang Meju Fermentation -Amino acids of oligopeptides formed during Chung-Kook-Jang Fermentation- (청국장(淸國醬)메주 발효과정중(醱酵過程中)의 질소화합물(望素化合物)의 소장(消長)에 관(關)한 연구(硏究)(II) -저급(低級) peptide와 구성(構成)아미노산(酸)에 관(關)하여-)

  • Park, Ke-In
    • Applied Biological Chemistry
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    • v.15 no.2
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    • pp.111-142
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    • 1972
  • An experimental Chung-Kook-Jang was prepared using the strain Bacillus subtilis sp. isolated by the author Samples were taken in 12 hrs interval during the fermentation and the oligopeptides were separated by the method of molecular sieving using the ion exchange resin column of Dowex-50. Only the X-16 fraction of oligopeptides was taken and the components of oligopeptides were developed in two dimensional thin layer chromatograms. The each peptide spot was eluted and each peptide was isolated. The pattern and kinds of amino acids, and N and C-terminal amino acids were studied. Fourteen different oligopeptides could be detected by the two dimensional thin layer chromatography, all of which were consisted of $4{\sim}9$ kinds of amino acids. No dipeptides and no tripeptides could be found. The N and C-terminal amino acids and the residual component amino acids of all these 14 peptides could be summarized as the follows. [P]-I. Pro (Cys Ala Asp Trp Ile Val) Glu [P]-II. Val (His Arg Glu Thr Ala Met) Asp [P]-III. Glu (Cys Lys Asp Thr Met) Ala [P]-IV. Glu(His Ser Ala) Met) [P]-V. Ile (Cys Asp Arg Gly Pro T.p Phe) His [P]-VI. Gly(Asp ser) Lys [P]-VII. Thr(Pro Tyr Phe) Asp [P]-VIII. Phe(Tyr Leu Ile) Val [P]-IX. Trp (Phelle) Thr [P]-X. Ile (Arg Leu) Phe [P]-XI. Asp (Lys His Ser Gly Glu Pro) Ala [P]-XII. Glu (Cys Asp Gly) Ser [P]-XIII. Ala (Arg Tyr) Glu [P]-XIV. Met (Glu Ala) His It appears that the protease of the Bacillus subtilis K-27 syrain has rather wider range of specificity than proteases of Aspergoillus soya, pepsin, chymotrypsin, and trypsin.

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Tectonic Movement in the Korean Peninsula (I): The Spatial Distribution of Tectonic Movement Identified by Terrain Analyses (한반도의 지반운동 ( I ): DEM 분석을 통한 지반운동의 공간적 분포 규명)

  • Park, Soo-Jin
    • Journal of the Korean Geographical Society
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    • v.42 no.3 s.120
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    • pp.368-387
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    • 2007
  • In order to explain geomorphological characteristics of the Korean Peninsula, it is necessary to understand the spatial distribution of tectonic movements and its causes. Even though geomorphological elements which might have been formed by tectonic movements(e.g. tilted overall landform, erosion surface, river terrace, marine terraces, etc.) have long been considered as main geomorphological research topics in Korea, the knowledge on the spatial distribution of tectonic movement is still limited. This research aims to identify the spatial distributions of tectonic movement via sequential analyses of Digital Elevation Model(DEM). This paper first developed a set of terrain analysis techniques derived from theoretical interrelationships between tectonic uplifts and landsurface denudation processes. The terrain analyses used in this research assume that elevations along major drainage basin divides might preserve original landsurfaces(psuedo-landsuface) that were formed by tectonic movement with relatively little influence by denudation processes. Psuedo-landsurfaces derived from a DEM show clear spatial distribution patterns with distinct directional alignments. Lines connecting psuedo-landsufaces in a certain direction are defined as psuedo-landsurface axes, which are again categorized into two groups: the first is uplift psuedo-landsurface axes that indicate the axis of landmass uplift; and the second is denudational psuedo-landsurface axes that cross step-shaped pusedo-landsurfaces formed via surface denudation. In total, 13 axes of pusedo-landsurface are identified in the Korean Peninsula, which show distinct direction, length, and relative uplift rate. Judging from the distribution of psudo-landsurfaces and their axes, it is concluded that the Korean Peninsula ran be divided into four tectonic regions, which are named as the Northern Tectonic Region, Center Tectonic Region, Southern Tectonic Region, and East Sea Tectonic Region, respectively. The Northern Tectonic Region had experienced a regional uplift centered at the Kaema plateau, and the rate of uplift gradually decreased toward southern, western and eastern directions. The Center Tectonic Region shows an arch-shaped uplift. Its uplift rate is the highest along the East Sea and the rate decreases towards the Yellow sea. The Southern Tectonic Region shows an asymmetric uplift centered a line connecting Dukyu and Jiri Mountains in the middle of the region. The eastern side of the Southern Regions shows higher uplift rate than that of the western side. The East Sea Tectonic Region includes south-eastern coastal area of the peninsula and Gilju-Myeongchun Jigudae, which shows relatively recent tectonic movements in Korea. Since this research visualizes the spatial heterogeneity of long-term tenonic movement in the Korean peninsula, this would provide valuable basic information on long-term and regional differences of geomorphological evolutionary processes and regional geomorphological differences of the Korean Peninsula.