• 제목/요약/키워드: Protein phosphatase 2A

검색결과 518건 처리시간 0.026초

A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth

  • Heo, Ju Sun;Choi, Ka Young;Sohn, Se Hyoung;Kim, Curie;Kim, Yoon Joo;Shin, Seung Han;Lee, Jae Myung;Lee, Juyoung;Sohn, Jin A;Lim, Byung Chan;Lee, Jin A;Choi, Chang Won;Kim, Ee-Kyung;Kim, Han-Suk;Kim, Beyong Il;Choi, Jung-Hwan
    • Clinical and Experimental Pediatrics
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    • 제55권11호
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    • pp.438-444
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    • 2012
  • Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

다슬기 protamex 가수분해물(MPH)의 항당뇨 기작 연구 (Anti-diabetic mechanism of melania snail (Semisulcospira libertina) protamex hydrolysates)

  • 표상은;최재석;김미령
    • 한국식품저장유통학회지
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    • 제24권7호
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    • pp.1007-1016
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    • 2017
  • 다슬기는 예로부터 간염, 간경화, 지방간 등의 치료 및 개선에 이용되어 왔으며, 특히 소변불통, 소갈증(당뇨) 등의 약용으로 이용되어 왔다. 본 연구에서는 이러한 다슬기를 대상으로 항당뇨에 대한 효능을 과학적으로 검증하고 그 기작을 규명하고자 하였다. 먼저 다슬기의 생물학적 기능성을 높이기 위해 효소 가수분해를 실시하였으며, protamex에 의한 가수분해도는 10시간 후 약 43% 수준을 나타내었다. PTP1B는 인슐린 신호전달기전에서 IRS-1의 인산화를 방해하여 인슐린 민감성을 저해시키는 효소이다. protamex를 이용한 다슬기 가수분해물(MPH)의 PTP1B에 대한 저해활성은 $15.42{\pm}1.1{\mu}g/mL$$IC_{50}$ 값을 나타내어 양성대조군 ursolic acid의 $16.7{\mu}g/mL$ 보다 높은 저해활성을 보이면서 강한 항당뇨 활성 소재로서의 가능성을 보였다. 이에 따라 유리지방산을 이용하여 C2C12 myoblast에서 인슐린 저항성을 유도하고, MPH에 의한 포도당 흡수 정도를 확인하였다. 그 결과, 1 mM PA 처리에 의해 약 32% 수준으로 떨어진 포도당 흡수율은 MPH 처리에 의해 약 199% 수준으로 증가하였다. 또한 장기간 고농도의 포도당(30 mM)에 의해 유도된 당독성 조건에서 MPH는 췌장의 베타세포 INS-1 세포의 생존율을 증가시키고, 대조군에 비해 약 160% 인슐린 mRNA 발현량을 증가시켰다. 이러한 결과에서 MPH는 PTP1B 활성을 저해함으로써 인슐린 신호전달 기작을 활성화하고, 인슐린저항성 환경에서 포도당 흡수를 증진시켜 인슐린저항성을 개선하며, 나아가 고농도 포도당에 의해 유도되는 당독성환경에서 췌장 베타세포를 보호하고 인슐린 mRNA발현량을 정상화할 수 있다는 것을 확인할 수 있었다.

인간치수세포에서 상아모세포의 분화과정 동안 Lysyl Oxidase Family의 역할 (Role of Lysyl Oxidase Family during Odontoblastic Differentiation of Human Dental Pulp Cells Induced with Odontogenic Supplement)

  • 이화정;한수연
    • 치위생과학회지
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    • 제13권3호
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    • pp.296-303
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    • 2013
  • LOX는 결합조직의 세포외기질에 존재하여 교원섬유와 탄성섬유의 교차결합을 촉진시키는 핵심적인 효소이다. 뼈세포에서 세포분화 및 세포이동과 관련된 LOX의 역할은 보고되었으나, 상아모세포 분화에서 LOX에 대한 직접적인 효과는 거의 알려지지 않았다. 이 연구에서는 인간치수세포에서 상아모세의 분화과정 동안 LOX의 역할에 대해 실험하였다. 치수세포에 저 혈청 분화유도 배지를 처리하여 0, 1, 3, 7, 14일 동안 배양하였다. 세포성장은 세포계수, 분화와 관련된 유전자들은 RT-PCR, 광물화는 alrizarin red S 염색으로 각각 실험하였다. LOX 유전자 발현은 RT-PCR로 측정하였고, LOX 효소활성은 고감도 형광분석을 시행하였다. 1. 세포성장은 저 혈청 분화유도 배지에서 시간 의존적으로 증가하였다. 2. 분화표지자인 ALP, OPN, OCN, DMP1, DSPP는 시간 의존적으로 발현되었으나 초기, 중기, 말기에 대한 차별화는 이루어지지 않았다. 3. 광물화는 3일부터 관찰되기 시작하여 14일까지 증가되었다. 4. LOX와 LOXL은 mRNA 발현이 7일까지 증가되었고, 14일에서 큰 감소를 보였다. 하지만, LOXL3와 LOXL4 mRNA는 낮은 발현을 보였고, LOXL2 mRNA는 발현되지않았다. 5. Collagen type I mRNA는 7일까지 증가하다가 14일에서 의미있게 감소를 보였고, collagen type IV는 낮은 mRNA 발현을 보였다. 6. LOX 효소 활성은 분화유도기간 중 7일에서 가장 많은 발현을 보였고, 교원질 1형 기질이 교원질 IV형 기질보다 두드러진 발현을 보였다. 이상의 결과를 종합해 볼 때, LOX isoform의 발현과 LOX 효소 활성은 인간치수세포에서 상아모세포 분화과정 동안 중요한 조절자로 사료된다.

삼채의 급여가 육계의 성장 및 혈액 생화학 특성에 미치는 영향 (Effects of Dietary Allium Hookeri on Growth and Blood Biochemical Parameters in Broiler Chickens)

  • 이은별;이선혜;김승환;강상현;이경우;김다혜;김동욱;강환구;김남석;김정봉;최정숙;장환희;황유진;김유석;이성현
    • 생약학회지
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    • 제49권2호
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    • pp.132-137
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    • 2018
  • This study was conducted to investigate the effects of dietary Allium hookeri on growth performance, bone strength, and blood biochemical profiles in growing broiler chickens. Twelve hundreds of one-day old Arbor Acres male broilers were divided into 6 treatments with 4 replicates and 50 birds per replicate (n=200 chicks/treatment). Chickens fed basal diet (Control), basal diet with commercial X (Positive control) at 0.05% of diet, or each one of the experimental diets (L3, L5, R3, R5) supplemented with the powder of A. hookeri leaf or root at 0.3 and 0.5% of diet respectively for 5 weeks. At the 5th week of feeding the diets, body weight, tibia strength, and blood biochemical profiles including antibody titers were measured. Dietary A. hookeri (L3, L5, R3, R5) significantly increased final body weight than the control group. And the dietary leaf of A. hookeri effectively increased the growth performance than dietary root of A. hookeri. Interestingly dietary leaf of A. hookeri improved tibia strength than the control group and L3 showed the highest value. The antibody titers against infectious bursal disease (IBD) increased with the addition of dietary leaf of A. hookeri compared with positive control, R3, and R5 groups. But there was no significant difference in serum biochemical parameters such as albumin, globulin, glucose, cholesterol, Ca, P, total protein, total bilirubin, alanine aminotransferase, alkaline phosphatase, and gamma-glutamyl transferase. These results suggest that A. hookeri can be used as a good supplement to improve growth performance and health by increasing bone strength and antibody titer against IBD without any anti-nutritional or toxic effects in growing broilers.

초음파를 이용한 간 섬유화 스캔 검사의 융합 탄성도 측정 평가 (Evaluation of convergence Elasticity of Liver Fibroscan used measurement with Ultrasonography)

  • 김민정;한만석;장재욱
    • 한국융합학회논문지
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    • 제8권5호
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    • pp.79-85
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    • 2017
  • 본 연구는 만성 B형 바이러스성 감염 환자의 간 기능 혈액 검사 수치와 간 섬유화 스캔 검사(FibroScan(R))의 비교 분석을 통한 간 섬유화 스캔 검사의 임상적, 기기적 융합 유용성을 평가하고자 하였다. 2015년 7월 1일부터 2016년 2월 28일까지 대전시 B내과에 내원한 만성 B형 바이러스성 감염 환자 75명의 간 섬유화 스캔 검사 결과와 간 기능 혈액검사 수치를 분석 하고 ROC곡선을 작성하였다. 알라닌 아미노전이요소, 아스파르테이트 아미노전이요소 수치가 각각 0.572, 0.502로 섬유화 수치와 가장 높은 상관관계를 보였다(p<0.000). 감마지티피, 총 빌리루빈, 알칼리성 인산분해효소 수치도 비교적 유의한 상관관계를 보였으나 알파태아단백과 총 단백정량은 통계적으로 유의하지 않았다. 또한 알부민(-0.449)과 혈소판치(-0.373)도 섬유화 수치와 상관관계가 없었다(p<0.000). 간 섬유화 정도가 높은 등급일수록 ROC 곡선의 정확도가 증가하였으며 F4의 간 경변 단계에서 가장 큰 AUROC값을 나타냈다. 따라서 간 섬유화 스캔 검사는 만성 간질환 환자의 간 기능 수치인 알라닌 아미노전이요소, 아스파르테이트 아미노전이요소 수치와 유의한 상관관계를 보여 간의 염증 검사 및 만성 간질환 진단에 매우 유용한 것으로 판단된다.

임상화학검사실에서 회수율 실험의 실증적 연구 (An Empirical Study of the Recovery Experiment in Clinical Chemistry)

  • 장상우;이상곤;송은영;박용원;박병옥
    • 대한임상검사과학회지
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    • 제38권3호
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    • pp.184-188
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    • 2006
  • The purpose of the recovery experiment in clinical chemistry is performed to estimate proportional systematic error. We must know all measurements have some error margin in measuring analytical performance. Proportional systematic error is the type of error whose magnitude increases as the concentration of analyte increases. This error is often caused by a substance in the sample matrix that reacts with the sought for analyte and therefore competes with the analytical reagent. Recovery experiments, therefore, are used rather selectively and do not have a high priority when another analytical method is available for comparison purposes. They may still be useful to help understand the nature of any bias revealed in the comparison of kit experiments. Recovery should be expressed as a percentage because the experimental objective is to estimate proportional systematic error, which is a percentage type of error. Good recovery is 100.0%. The difference between 100 and the observed recovery(in percent) is the proportional systematic error. We calculated the amount of analyte added by multiplying the concentration of the analyte added solution by the dilution factor(mL standard)/(mL standard + mL specimen) and took the difference between the sample with addition and the sample with dilution. When making judgments on method performance, the observed that the errors should be compared to the defined allowable error. The average recovery needs to be converted to proportional error(100%/Recovery) and then compared to an analytical quality requirement expressed in percent. The results of recovery experiments were total protein(101.4%), albumin(97.4%), total bilirubin(104%), alkaline phosphatase(89.1%), aspartate aminotransferase(102.8), alanine aminotransferase(103.2), gamma glutamyl transpeptidase(97.6%), creatine kinase(105.4%), lactate dehydrogenase(95.9%), creatinine(103.1%), blood urea nitrogen(102.9%), uric acid(106.4%), total cholesterol(108.5), triglycerides(89.6%), glucose(93%), amylase(109.8), calcium(102.8), inorganic phosphorus(106.3%). We then compared the observed error to the amount of error allowable for the test. There were no items beyond the CLIA criterion for acceptable performance.

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Effect of Microbial Phytase on Performance, Nutrient Absorption and Excretion in Weaned Pigs and Apparent Ileal Nutrient Digestibility in Growing Pigs

  • Zeng, Z.K.;Piao, X.S.;Wang, D.;Li, P.F.;Xue, L.F.;Salmon, Lorraine;Zhang, H.Y.;Han, X.;Liu, L.
    • Asian-Australasian Journal of Animal Sciences
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    • 제24권8호
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    • pp.1164-1172
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    • 2011
  • Two experiments were conducted to evaluate the efficacy of Trichoderma reesei derived phytase for pigs fed diets with fixed calcium to total phosphorus ratios (1.5:1). In Exp. 1, 280 weaned pigs (initial BW of $10.32{\pm}1.94$ kg) were allocated to one of five dietary treatments on the basis of weight and gender in a randomized complete block design. Treatments were the low phosphorus (0.6% Ca, 0.4% total P and 0.23% available P) diets supplemented with 0, 250, 1,000, or 2,000 FTU phytase/kg of diet and a positive control diet (PC; 0.85% Ca, 0.58% total P and 0.37% available P). The treatments were applied to seven pens with eight pigs per pen, half male and half female. In Exp. 2, six barrows fitted with ileal T-cannula (initial BW = $35.1{\pm}1.6$ kg) were assigned to three dietary treatments with a double $3{\times}3$ Latin square design. The dietary treatments were the low-phosphorus diet (0.53% Ca, 0.34% total P and 0.14% available P), the low phosphorus diet plus 1,000 FTU phytase/kg and a positive control diet (0.77% Ca, 0.50% total P and 0.30% available P). In Exp. 1, there were linear increases (p<0.01) in weight gain, phosphorus absorption, bone strength, calcium and phosphorus content of fat-free dried bone and plasma phosphorus concentrations with increasing dose rate of phytase. The performance of pigs fed the diets with 250, 1,000, or 2,000 FTU of phytase/kg did not differ from pigs fed the PC diet. Pigs fed diets with 1,000 or 2,000 FTU of phytase/kg did not differ from pigs fed the PC diet in bone characteristics. The apparent digestibility of dry matter, crude protein, ash and energy was not affected by dietary treatment. However, pigs fed the PC diet excreted more fecal phosphorus (g/d, p<0.01) and fecal phosphorus per BW gain (g/kg) than pigs fed the diets with phytase. Phytase linearly decreased (p<0.01) fecal phosphorus excreted per BW gain (g/kg), plasma calcium concentration as well as plasma and bone alkaline phosphatase activity. In Exp. 2, phytase supplementation in the low-P diet increased (p<0.05) the apparent ileal digestibility (AID) of Ca, P, leucine, lysine, phenylalanine, alanine and cysteine, tended to AID of crude protein, isoleucine, threonine, asparagine and serine. In conclusion, the novel phytase originated from Trichoderma reesei is effective in releasing Ca, P, and amino acids from corn soy based diet for pigs.

신령버섯 균사체 액체배양액이 고지방 식이를 급여한 흰쥐의 지질대사 및 효소활성에 미치는 영향 (Effects of Liquid Culture of Agaricus blazei Murill on Lipid Metabolism and Enzyme Activities in Rats Fed High Fat Diet)

  • 이현정;고진복
    • Journal of Nutrition and Health
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    • 제36권4호
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    • pp.352-358
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    • 2003
  • 신령버섯 균사체 배양액이 고지방식이를 섭취한 숫쥐의 성장률, 장기무게, 지질 농도, 단백질 농도 및 효소활성에 미치는 영향을 조사하고자, 생후 7주령의 흰쥐 (255.3 $\pm$ 20.2 g)에 표준식이를 급여한 정상군, 표준식이에 15% 돈지를 첨가한 식이를 급여한 고지방군, 고지방 식이에 신령버섯의 균사체 배양액을 음료수에 20% 및 30%로 혼합 급여한 군 (20% 및 30% 신령버석군) 등 4군으로 나누어 5주간 사육한 결과는 다음과 같다. 실험동물의 체중증가량, 식이섭취량 및 식이효율, 그리고 간, 신장, 비장 및 췌장의 무게는 고지방군과 20% 및 30% 신령버섯군이 비슷한 수준을 유지하였다. 부고환지방은 정상군에 비하여 고지방군 및 신령버섯군들이 유의하게 증가되어 신령버섯 섭취에 따른 감소효과는 나타나지 않았다. 혈청의 총 콜레스테롤, 중성지질, LDL-콜레스테롤, HDL-콜레스테를 농도 및 glutamic pyruvic transaminase 활성은 고지방군에 비해 30%신령버섯군이 유의하게 감소되었다. 간의 콜레스테롤과 중성지질 농도는 고지방군과 20% 및 30% 신령버섯군이 비슷한 수준으로 감소효과가 나타나지 않았다. 총 콜레스테롤에 대한HDL-콜레스테롤의 비율은 고지방군에 비해 30%신령버섯군이 유의하게 증가되었다. 혈청의 단백질, 혈색소 및 혈당 농도, 동맥경화지수와 alkaline phosphatase 활성은 정상군과 각 실험군이 비슷한 수준을 유지하였다. 혈청의 glutamic oxaloacetic transaminase 활성은 고지방군과 20% 및 30% 신령버섯군이 비슷한 경향이었다. 이상의 결과로 보아 고지방식이를 급여한 흰쥐에 신령버섯 균사체 배양액을 30% 급여시 체중증가량과 장기 무게는 정상수준을 유지하였고, 혈청의 총 콜레스테롤, 중성지질 및 LDL-콜레스테를 농도를 낮추고, 총 콜레스테롤에 대한 HDL-콜레스테롤의 비율을 증가시키는 효과가 나타났다.

골아세포의 IGF-I 유전자 발현 및 세포증식에 대한 1,25-dihydroxyvitamin $D_3$의 영향 (The Effects of 1,25- Dihydroxyvitamin $D_3$ on Expression of IGF-I Gene and Cellular Proliferation in MC3T3-E1 Cells)

  • 최희동;이재목;서조영
    • Journal of Periodontal and Implant Science
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    • 제30권1호
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    • pp.39-52
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    • 2000
  • Polypeptide growth factor belong to a class of potent biologic mediator which regulate cell differentiation, proliferation, migration and metabolism. 1,25-dihydroxyvitamin $D_3$ decrease cell proliferation, and stimulate alkaline phosphatase activity which express in osteoblast during cell differentiation period. IGF-I is known to stimulate cell proliferation and differentiation too. 1,25-dihydroxyvitamin $D_3$ is known to increase IGF-I binding sites and IGF binding protein which inhibite the effect of IGF. The purpose of this study is to evaluate potential role of IGF-I as mediator that control the action of 1,25-dihydroxyvitamin $D_3$. MC3T3-E1 cell were seeded $5{\times}10^5/ml$ at 100mm culture plate in ${\alpha}-MEM$ containing 10% fetal bovine serum. After 48 hour incubation period, medium were changed ${\alpha}-MEM$ containing 5% fetal bovine serum. After 24 hours, $10^{-9}M$ 1,25-dihydroxyvitamin $D_3$ added. Total mRNA was extracted at 0, 6, 24, 48, 72 hour. PRPCR method was programed for the detection of IGF-I mRNA. In the both groups of 1,25-dihydroxy vitamin $D_3$ treated and control, alternative splicing form of IGF-I, IGF-IA and IGF-IB were expressed. In the 1,25-dihydroxyvitamin $D_3$ treated group, IGF-I mRNA expression was matained until 24 hour, there after expression was decresed. MC3T3-E1 cell were seeded $2.5{\times}10^4/ml$ at 24well plate in ${\alpha}-MEM$ containing 10% fetal bovine serum. After 48 hour incubation period, medium were changed ${\alpha}-MEM$ containing 3% fetal bovine serum. After 24 hours, $10^{-9}M$ 1,25-dihydroxyvitamin $D_3$ and 10 ng/ml IGF-I were added separately or together. Cell were cultured for 1 and 3 days, $2{\mu}Ci/ml\;[^3H]$ -thymidine was added for the last 24h of culture of each days. ${[^3H]}$-thymidine incorporation in to DNA was measured and expressed counter per minute(CPM). DNA synthetic activity was significantly decreased by 1,25-dihydroxyvitamin $D_3$ both at 1 day and 3 day, and in the combination group of 1,25-dihydroxyvitamin $D_3$ and IGF-I, DNA synthetic activity was also decreased both at 1 day and 3 days. IGF-I did not affect the DNA synthetic activity compared to control group both at 1 day and 3 day. From the above results, 1,25-dihydroxyvitamin $D_3$ was potent inhibitor of cell proliferaton in MC3T3-E1 cells. It assumed that the effect of 1,25-dihydroxyvitamin $D_3$ on osteoblast proliferation may be mediated in part by decreased level of IGF-I.

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Effects of absorbents on growth performance, blood profiles and liver gene expression in broilers fed diets naturally contaminated with aflatoxin

  • Liu, J.B.;Yan, H.L.;Cao, S.C.;Hu, Y.D.;Zhang, H.F.
    • Asian-Australasian Journal of Animal Sciences
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    • 제33권2호
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    • pp.294-304
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    • 2020
  • Objective: The study was conducted to evaluate the effects of the absorbent (a mixture of activated carbon and hydrated sodium calcium aluminosilicate) on growth performance, blood profiles and hepatic genes expression in broilers fed diets naturally contaminated with aflatoxin. Methods: A total of 1,200 one-day-old male chicks were randomly assigned to 6 treatments with 10 replicate cages per treatment. The dietary treatments were as follows: i) control (basal diets); ii) 50% contaminated corn; iii) 100% contaminated corn; iv) control+1% adsorbent; v) 50% contaminated corn+1% absorbent; vi) 100% contaminated corn+1% absorbent. Results: During d 1 to 21, feeding contaminated diets reduced (p<0.05) body weight (BW), average daily gain (ADG), and average daily feed intake (ADFI), but increased (p<0.05) feed-to-gain ratio (F/G). The absorbent supplementation increased (p<0.05) BW, ADG, and ADFI. There were interactions (p<0.05) in BW, ADG, and ADFI between contaminated corn and absorbent. Overall, birds fed 100% contaminated diets had lower (p<0.05) final BW and ADG, but higher (p<0.05) F/G compared to those fed control diets. The absorbent addition increased (p<0.05) serum albumin concentration on d 14 and 28 and total protein (TP) level on d 28, decreased (p<0.05) alanine transaminase activity on d 14 and activities of aspartate aminotransferase and alkaline phosphatase on d 28. Feeding contaminated diets reduced (p<0.05) hepatic TP content on d 28 and 42. The contaminated diets upregulated (p<0.05) expression of interleukin-6, catalase (CAT), and superoxide dismutase (SOD), but downregulated (p<0.05) glutathione S-transferase (GST) expression in liver. The absorbent supplementation increased (p<0.05) interleukin-1β, CAT, SOD, cytochrome P450 1A1 and GST expression in liver. There were interactions (p<0.05) in the expression of hepatic CAT, SOD, and GST between contaminated corn and absorbent. Conclusion: The results suggest that the naturally aflatoxin-contaminated corn depressed growth performance, while the adsorbent could partially attenuate the adverse effects of aflatoxin on growth performance, blood profiles and hepatic genes expression in broilers.