• 제목/요약/키워드: C7 deficiency

검색결과 163건 처리시간 0.033초

임신 중기의 아연 영양상태가 임신결과에 미치는 영향 (Zinc Status of South Korean Women in the Second Trimester and the Effect of Those on Pregnancy Outcome)

  • 이현숙
    • Journal of Nutrition and Health
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    • 제38권7호
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    • pp.578-585
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    • 2005
  • Maternal zinc deficiency is relatively common worldwide, but its consequences for pregnancy outcome are not established. The purpose of this study was to examine the effect of zinc status in the second trimester on pregnancy outcome. Subjects were 248 pregnant Korean women (25-28 wk gestation). Cord blood was collected from subgroup of 69 women and pregnancy outcome data were obtained from 185 babies. Anthropometry measurement, dietary intake, and biochemical characteristics of pregnancy and cord serum substances were measured. The subjects were divided into quartiles on the basis of maternal serum Zn concentration; $ZnQ_1$ (< 25 percentile), $ZnQ_2$ ($26\~50$ percentile), $ZnQ_3$ ($51\~75$ percentile), and $ZnQ_4$ ($76\~100$ percentile). Zn groups were compared in terms of various maternal factors, concentrations of cord serum substances, and pregnancy outcome. Maternal serum Zn concentration was $118.4\;{\pm}\;35.5\;{\mu}g/dl$ as mean. Intake of Zn was lower than Korean RDA. The rate of Zn deficiency among all subjects was $8.5\%$. Maternal serum Zn levels belonged to normal range. Cord serum Zn level was about $154.7\%$ of maternal serum level. Intakes of energy, calcium, iron, folic acid, and riboflavin did not meet the Korean RDA for pregnant women by gestational age. The mean birth weight of neonates is 3083 $\pm$ 697 g, of whom $9.1\%$ were of low birth weight (< 2,500 g). Maternal serum Zn level was positively correlated with pre-pregnancy weight, pre-pregnancy body mass index, and vitamin C intake (p < 0.05). $ZnQ_1$ group had significantly lower maternal serum iron concentration and higher cord serum cholesterol than those in other groups. Maternal serum Zn level, cord serum Zn level, and dietary Zn intake were no related to the pregnancy outcome. The birth weight had a correlation with the maternal hemoglobin and albumin concentration. In conclusion, at this study, we could not find the association with maternal Zn status in 2nd trimester and pregnancy outcome.

SAW 필터용 ZnO 압전 박막의 전기적 특성 (Electrical Characteristics of ZnO Piezo-electric Thin film for SAW filter)

  • 이동윤;윤석진
    • 한국전기전자재료학회논문지
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    • 제18권10호
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    • pp.909-916
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    • 2005
  • The structural and electrical property of RF magnetron sputtered ZnO thin film have been studied as a function of RF power, substrate temperature, oxygen/argon gas ratio and film thickness at constant sputtering power, sputtering working pressure and target-substrate distance. To analyze a crystallo-graphic properties of the films, $\theta$/2$\theta$ mode X-ray diffraction, SEM, and AFM analyses. C-axis preferred orientation, resistivity and surface roughness highly depended on oxygen/argon gas ratio. The resistivity of ZnO thin film(6000 ${\AA}$) rapidly increased with increasing oxygen ratio and the resistivity value of $9 {\ast} 10^7 {\Omega}cm$ was obtained at a working pressure of 10 mTorr with the same oxygen/argon gas ratio. The surface roughness was also improved with increasing oxygen ratio and the ZnO films deposited with the same oxygen/argon gas ratio showed the excellent roughness value of 28.7 ${\AA}$. With increase of the substrate temperature, The C-axis preferred orientation of ZnO thin film increases and the resistivity decreases due to deviation from the stoichiometric ZnO due to oxygen deficiency.

Duarte Variant/Classical Galactosemia (D/G) Heterozygote으로 진단된 일란성 쌍둥이 1례 (A Twin diagnosed with Duarte Variant/Classical (D/G) Galactosemia)

  • 구교연;이철호;양정윤;이진성
    • 대한유전성대사질환학회지
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    • 제12권1호
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    • pp.58-63
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    • 2012
  • 고전적 갈락토오스혈증(classical galactosemia; OMIM #230400)은 상염색체 열성 유전의 갈락토오스 대사장애로, 9번 염색체에 위치하는 GALT 유전자(OMIM *606999)로부터 전사되는 galactose-1-phosphate uridylyltransferase (GALT; E.C.2.7.7.12)의 심각한 결손으로 인해 유발되는 질환이다. GALT의 결함은 galactose-1-phosphate의 체내 축적을 일으켜, 신생아 시기부터 구토, 수유 곤란, 황달, 복수, 경련 발작, 기면 상태 등의 증상을 유발하고, 장기적으로는 백내장, 성장지연, 지능저하를 초래한다. 반면 Duarte형 갈락토오스혈증은 적혈구에서의 GALT 효소 활성도가 감소되어 있어 혈중 galactose와 galactose-1-phosphate의 농도가 증가하지만 임상적으로는 거의 증상을 보이지 않는 아형이다. 최근 신생아 선별검사의 발달과 함께, 무증상의 양성 판정 환아들이 늘고 있으며, 이들 환아들이 Duarte형 갈락토오스혈증일 가능성이 제기되고 있다. 이에 저자들은 국내에서 그 동안 드물게 보고되었던 N314D와 -119_-116delGT CA/E363K이형접합체의 Duarte형 갈락토오스혈증 일란성 쌍둥이 1례를 보고하고자 한다.

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난소 과자극증후군과 동반된 뇌바닥동맥 혈전증 (A Case of Basilar Arterial Thrombosis in Ovarian Hyperstimulation Syndrome)

  • 박준철;임수연;배진곤;김종인;이정호
    • Clinical and Experimental Reproductive Medicine
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    • 제35권1호
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    • pp.83-88
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    • 2008
  • 보조생식술 시행 후 혈전색전증의 발생은 매우 드물기는 하나 치명적인 합병증이다. 과배란 유도시 중증 난소 과자극 증후군은 $0.1{\sim}0.5%$에서 발생하며, 중증 난소 과자극증후군 환자의 128명 중 한 명에서 혈전색전증이 발생하는 것으로 알려져 있다. 발병기전으로는 과배란 유도에 따른 고에스트로젠 혈증에 의한 혈액응고인자의 변화, 혈관 투과성의 증가에 따른 혈액농축 및 순환혈액의 감소 등을 원인으로 추정하고 있으나 그 정확한 기전은 알려진 바가 없다. 또한 thrombophilia나, 혈전색전증의 과거력 및 가족력이 있는 경우 발병율이 증가한다. 혈전증의 발생 부위는 정맥이 67%이나, 33%는 동맥에 발병하며 주로 뇌동맥에 발생된다고 보고되었다. 본 예는 3년간의 이차성 불임을 주소로 내원하여 시험관 아기시술을 시행하였으며 hCG 투여 8일 후 난소 과자극증후군이 중등도로 발생하였다. hCG 투여 후 11일째 갑작스런 의식 소실 및 우측 상지의 운동장애가 발생하여 시행한 MRI상 뇌바닥동맥 (basilar a.) 혈전증으로 진단되었으며, 혈관내 혈전 용해술 및 풍선 확장술 시행후 재관류에 성공하였다. 시술 2일 후 의식 및 운동장애는 완전히 회복되었으며 시술 7일 후 말더듬증 역시 회복되어 후유장애 없이 건강한 상태이다. Thrombophilic study상 Protein S결핍 소견을 보였고, 또한 vWF-associated Ag.이 증가된 양상을 보였다. 지금까지 국내에서 난소 과자극증후군 이후에 발생한 뇌경색은 5예가 보고되었으며, 이중 4예에서 protein S deficiency 소견을 보였으므로 선별 검사로 유용할 것으로 사료된다. 저자 등은 3년간의 이차성 불임을 주소로 내원한 33세 환자에서 시험관 아기시술시 hCG 투여 11일째에 발생한 뇌바닥동맥 혈전증을 간단한 문헌 고찰과 함께 보고하는 바이다.

10 year follow up of a boy with Lesch Nyhan Syndrome

  • Kim, Sook Za;Song, Wung Ju
    • 대한유전성대사질환학회지
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    • 제11권1호
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    • pp.88-98
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    • 2011
  • Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.

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YBCO 고온 초전도체 후막의 제작 및 특성 연구 (Fabrication and Characterization of High $T_c$ $YBa_2Cu_3O_7-x$ Thick Films)

  • 정형진;박홍순;이전국;송진태
    • 한국세라믹학회지
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    • 제27권3호
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    • pp.299-310
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    • 1990
  • The fabrication of Y-Ba-Cu-O films and experimental factors affecting to characteristics of films are studied in detail. Superconducting thick films are fabricated by the screen printing method. The metal-citrate precursors are obtained simply by mixing of metal nitrate with citric acid. The particle size of heat treated powders is approximately 0.3-1${\mu}{\textrm}{m}$ and the thickness of film is estimated as 30-35${\mu}{\textrm}{m}$. According to the XRD analysis, the films sintered at 870-93$0^{\circ}C$ for 10min. -6hr with P(oxygen)=1atm has the unique orthorhombic crystal symmetry indicating the 123 phae. Tc, onset is determined around 92-87。K, but it shows semiconducting behavior probably due to the oxygen deficiency in the lattice, porosity and impurities in films. Extrapolated Tc, zero is estimated as 76-50。K. For films sintered at 90$0^{\circ}C$ for 1hr, superconducting properties are observed with the room temperature resistivity of 0.025$\Omega$.cm Tc, onset at 88。K and Tc, zero at 63。K.

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마곡지구 하수열에너지이용 타당성 검토 (Feasibility study on district heating Magok area by sewage water heat source)

  • 이영수
    • 대한설비공학회:학술대회논문집
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    • 대한설비공학회 2009년도 하계학술발표대회 논문집
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    • pp.357-362
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    • 2009
  • As a unutilized energy, treated sewage water locates widespread near urban areas. From the previous survey, the sewage water is reported to hold energy potential up to 36,000 Tcal/year, which was 2.1% of the total domestic energy consumption and 9.7% of the energy usage in the household and business sector in 2006. Temperature of the sewage water differs locally, but its range is observed in a range of $20{\sim}25^{\circ}C$ in summer and $8{\sim}13^{\circ}C$ in winter. Since the temperature range of the sewage water has a better seasonal distribution about $5{\sim}10^{\circ}C$ compared to ambient air, it is a promising heat sink for summer or heat source for winter. The sewage water is also a high quality heat source from its abundant quantity and uniform temperature. Considering the ambient temperature of Korea is very low in winter, a heat pump system using the sewage water can be an alternative to prevent problems of capacity deficiency and frost formation.

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Evaluation of Antioxidant Status and Correlation among Antioxidant Indices in Female College Students

  • Kim, Jung-Hee;Heajoon Ahn
    • Journal of Community Nutrition
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    • 제5권1호
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    • pp.13-20
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    • 2003
  • This study was done to evaluate the antioxidant status of female college students by determining their intakes and plasma levels of antioxidnt vitamins (vitamin C, A and E) and total antioxidant status (TAS). Subjects were 46 healthy female college students aged 20 - 29 years. Body composition was determined by a multifrequency bioelectrical impedance analysis. Dietary intakes were examined by 24hr record method and nutrients intakes were analyzed by the Computer Aided Nutritional analysis program for professional (CAN-pro). Plasma vitamin C level were measured by spectrophotometric method and retinol, ${\beta}$-carotene, ${\alpha}$-tocopherol were measured by HPLC. Plasma TAS was measured with a Randox kit using the trolox equivalent antioxidant capacity (TEAC) method. Daily energy and protein intakes of the female college students were 1670.5㎉ (83% of RDA) and 63.3g (115.1% of RDA), respectively. However their intakes of Ca and Fe were below 75% of RDA. Their intakes of vitamin A and C were 596.6 ${\mu}$ gRE (85.2% of RDA) and 71.0mg (101.4% of RDA), respectively. Plasma levels of vitamin C, retinol, ${\beta}$-carotene and ${\alpha}$-tocopherol were 14.7mg/L, 0.7mg/L, 0.2mg/L and 9.1mg/L, respectively which were within normal range. There was no subject with deficiency or marginal level in plasma vitamin A and C. However 1.6% of the subjects had below adequate level in vitamin E. Plasma TAS level was 1.2mmol/L. Correlation data showed that all plasma antioxidant vitamins were positively correlated with plasma TAS. Overall data indicate that the antioxidant status of female college students were pretty good. However it might be necessary to educate them to eat more fruits and vegetables for preventing many chronic diseases in a later life. (J Community Nutrition 5(1) : 13∼20, 2003)

The multifunctional RNA-binding protein hnRNPK is critical for the proliferation and differentiation of myoblasts

  • Xu, Yongjie;Li, Rui;Zhang, Kaili;Wu, Wei;Wang, Suying;Zhang, Pengpeng;Xu, Haixia
    • BMB Reports
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    • 제51권7호
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    • pp.350-355
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    • 2018
  • HnRNPK is a multifunctional protein that participates in chromatin remodeling, transcription, RNA splicing, mRNA stability and translation. Here, we uncovered the function of hnRNPK in regulating the proliferation and differentiation of myoblasts. hnRNPK was mutated in the C2C12 myoblast cell line using the CRISPR/Cas9 system. A decreased proliferation rate was observed in hnRNPK-mutated cells, suggesting an impaired proliferation phenotype. Furthermore, increased G2/M phase, decreased S phase and increased sub-G1 phase cells were detected in the hnRNPK-mutated cell lines. The expression analysis of key cell cycle regulators indicated mRNA of Cyclin A2 was significantly increased in the mutant myoblasts compared to the control cells, while Cyclin B1, Cdc25b and Cdc25c were decreased sharply. In addition to the myoblast proliferation defect, the mutant cells exhibited defect in myotube formation. The myotube formation marker, myosin heavy chain (MHC), was decreased sharply in hnRNPK-mutated cells compared to control myoblasts during differentiation. The deficiency in hnRNPK also resulted in the repression of Myog expression, a key myogenic regulator during differentiation. Together, our data demonstrate that hnRNPK is required for myoblast proliferation and differentiation and may be an essential regulator of myoblast function.

배지 조성이 Haematococcus pluvialis의 생장과 Astaxanthin 생산에 미치는 영향 (Effects of Medium Copositions for the Growth and the Astaxanthin Production of Haematococcus pluvialis)

  • 박은경;서문원;이철균
    • 한국미생물·생명공학회지
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    • 제29권4호
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    • pp.227-233
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    • 2001
  • 고부가가치의 천연 astaxanthin의 생산을 위한 Haematococcus pluvialis의 고농도 배양을 수립하기 위하여 세포 농도를 증가시키는 방법과 세포 내 색소를 증가시키는 방법 두 가지에 초점을 맞춰 본 연구를 수행하였다 Hongkong Medium (HKM) Modifed Bolds Basal Medium (MBBM) Modified Bristols Medium(MBM) Proteose-petone Medium (PPM) BG-11 Medium의 여러 배지를 사용하여 비교함으로서 배지 내 탄소원 질소원과 미량원소 등이 세포의 생장과 astaxan-tin 생산에 미치는 영향을 보면 HKM 은 $2.0$\times$10^{6}$ cells /mL의 세포 농도와 최고 6.14 mg/g cell의 astaxanthin content per cell 로 다른 배지 중 가장 높게 나타나므로 세포 농도를 높이기위해서 질소우너이 220 mg/L 인 HKM이 적당하고 색소 형성을 촉진시키기 위해서는 aggregation이 일어나 단위 세포당 astaxanthin 함량이 9.7 mg/g cell 로 높게 나타나는 MBBM이 월등함을 알수 있었다. H. pluvialis의 최적 배양 온도와 pH를 선정하깅 nl하여 $20^{\circ}C$ $25^{\circ}C$, 3$0^{\circ}C$ 온도와 pH 4.5 6.0 7.5 의 배양조건을 수립한 결가 pH $7.5 20^{\circ}C$$25^{\circ}C$에서는 세포생장과 색소 형성이 우수함을 관찰 할 수 있었고 배양에 적당한 20 $^{\circ}C$ 에서 배양하다가 $30^{\circ}C$로 24시간 배양항 heating 효과를 주면 astaxanthin 축적을 촉진시키는데 효과적임을 알수있었다. PPMso의 질소원과 proteose-peptone의 고갈시세포의 생장의특성과 astaxanthin 생산에 미치는 영향은 control 배지에서 세포 농도와 세포의 건조질량이 각각 $0.98$\times$10^{6}$ / cell /mL 와 0.2 g/L astaxanthin의 농도는 1.92 mg/L 단위 세포당 astaxanthin 농도는 9.6 mg/g cell 로 관찰되었다결론적으로 질소원과 peptone이 고갈되면 세포의 생장은 억제되나 astaxanthin의 생산은 촉진됨을 알수 있었으며 세포 생장을 촉진하는 광도 60$\mu$E/($\m^2$s)와 HKM 배지 이용의 1단계와 높은 광도와 MBBM배지를 이용한 색소 생산의 2단계 배양을 최적조건으로 수립하였다.

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