• 제목/요약/키워드: Plasma amino acid

검색결과 217건 처리시간 0.025초

14년간 신생아 선별검사에서 고메티오닌혈증으로 전원된 환아들의 임상적 고찰 (Spectrum of patients with hypermethioninemia based on neonatal screening tests over 14 years)

  • 오세정;홍용희;이용화;이동환
    • Clinical and Experimental Pediatrics
    • /
    • 제53권3호
    • /
    • pp.329-334
    • /
    • 2010
  • 목 적 : 신생아 선별검사에서 고메티오닌혈증 소견을 보여 전원된 환아들의 혈액 및 소변 아미노산 분석과 호모시스테인 검사 결과를 통해 호모시스틴뇨증, 단독성 고메티오닌혈증, 일과성 고메티오닌혈증의 빈도를 알아보고 이들의 임상 양상에 대해 분석하고자 하였다. 방 법 : 1996년 1월부터 2009년 8월까지 신생아 선별검사상 고메티오닌혈증 또는 호모시스틴뇨증 의심 하에 본원으로 의뢰된 환아 58명을 대상으로 하였다. 각 환아의 입원 또는 외래 내원기록을 후향적으로 검토하여 혈액 및 소변 아미노산 분석 검사 결과, 혈장 호모시스테인(Homocysteine) 측정 결과 등을 조사하였다. 결 과 : 정밀검사 결과 분석을 통해 진단 기준에 따라 대상 환아 총 58명에서 28명(48.3%)가 정상으로 판명되었고 12명(20.7%)는 일과성으로 판명되었다. 호모시스틴뇨증과 단독성 고메티오닌혈증 환아들은 각각 3명(5.1%), 15명(25.9%)이었고 첫 내원시 평균 연령이 각 $50.0{\pm}22.5$일, $34.9{\pm}13.5$일이었으며 특수 분유와 식사 요법을 이용한 조기 치료 이후 현재까지 모두 정상 발달을 유지하고 있다. 결 론 : 신생아 선별검사에서 고메티오닌혈증을 보이는 경우 혈액과 소변 아미노산 분석을 시행함으로써, 치료하지 않을 경우 영구적인 기능 저하 및 발육 부진을 초래할 수 있는 호모시스틴뇨증 뿐만 아니라 비교적 양성 질환인 단독성 고메티오닌혈증을 진단할 수 있고 이러한 조기 진단과 치료를 통해 각 질환 환아의 정상적인 성장이 이루어질 수 있다는 점을 염두하여 이들의 조기 진단 및 추적 관찰을 시행해야 하겠다.

Effects of protein content and the inclusion of protein sources with different amino acid release dynamics on the nitrogen utilization of weaned piglets

  • Hu, Nianzhi;Shen, Zhiwen;Pan, Li;Qin, Guixin;Zhao, Yuan;Bao, Nan
    • Animal Bioscience
    • /
    • 제35권2호
    • /
    • pp.260-271
    • /
    • 2022
  • Objective: We aimed to investigate the effect of the differing amino acid (AA) release dynamics of two protein sources on the growth performance, nitrogen deposition, plasma biochemical parameters, and muscle synthesis and degradation of piglets when included in their diets at normal and low concentrations. Methods: Forty-eight piglets (Duroc×Landrace×Large White) with initial body weight of 7.45±0.58 kg were assigned to six groups and fed one of 6 diets. The 6 dietary treatments were arranged by 3×2 factorial with 3 protein sources and 2 dietary protein levels. They are NCAS (a normal protein content with casein), NBlend (a normal protein content with blend of casein and corn gluten meal), NCGM (a normal protein content with corn gluten meal), LCAS (a low protein content with casein), LBlend (a low protein content with blend of casein and corn gluten meal), LCGM (a low protein content with corn gluten meal). The release dynamics of AA in these diets were determined by in vitro digestion. The digestibility, utilization and biological value of nitrogen in piglets were determined by micro Kjeldahl method. Plasma insulin was measured by enzyme-linked immunosorbent assay kits. The protein expression of mediators of muscle synthesis and degradation was determined by western blotting. Results: Although the consumption of a low-protein diet supplemented with crystalline AA was associated with greater nitrogen digestion and utilization (p<0.05), the final body weight, growth performance, nitrogen deposition, and phosphorylation of ribosomal protein S6 kinase 1 and eIF4E binding protein 1 in the muscle of pigs in the low-protein diet-fed groups were lower than those of the normal-protein diet-fed groups (p<0.05) because of the absence of non-essential AA. Because of the more balanced release of AA, the casein (CAS) and Blend-fed groups showed superior growth performance, final body weight and nitrogen deposition, and lower expression of muscle ring finger 1 and muscle atrophy F-box than the CGM-fed groups (p<0.05). Conclusion: We conclude that the balanced release of AA from CAS containing diets and mixed diets could reduce muscle degradation, favor nitrogen retention, % intake and improve growth performance in pigs consuming either a normal- or low-protein diet.

Liver metabolic perturbations of heat-stressed lactating dairy cows

  • Fan, Caiyun;Su, Di;Tian, He;Li, Xiaojiao;Li, Yu;Ran, Lei;Hu, Ruiting;Cheng, Jianbo
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제31권8호
    • /
    • pp.1244-1251
    • /
    • 2018
  • Objective: The objective of the present study was to elucidate the mechanism underlying liver metabolic perturbations in dairy cows exposed to heat stress (HS). Methods: Liquid chromatography massabl spectrometry was used to analyze metabolic differences in livers of 20 dairy cows, with and without exposure to HS. Results: The results revealed 33 potential metabolite candidate biomarkers for the detection of HS in dairy cows. Fifteen of these metabolites (glucose, lactate, pyruvate, acetoacetate, ${\beta}$-hydroxybutyrate, fumaric acid, citric acid, choline, glycine, proline, isoleucine, leucine, urea, creatinine, and orotic acid) were previously found to be potential biomarkers of HS in plasma or milk, discriminating dairy cows with and without HS. Conclusion: All the potential diagnostic biomarkers were involved in glycolysis, amino acid, ketone, tricarboxylic acid, or nucleotide metabolism, indicating that HS mainly affected energy and nucleotide metabolism in lactating dairy cows.

유기산성약물이 푸로세미드의 흡수, 배설및 이뇨효과에 미치는 영향 (The Effects of Organic Acidic Drugs on the Absorption, Excretion and Diuretic Action of Furosemide)

  • 염윤희;용재익
    • 약학회지
    • /
    • 제31권5호
    • /
    • pp.273-279
    • /
    • 1987
  • The effects of organic acidic drugs on the absorption, excretion and diuretic action of furosemide were studied. Cefalexin, p-aminohippuric acid (PAH), ibuprofen and p-amino salicylic acid (PAS) were selected as organic acidic drugs. The in situ absorption rate and absorption rate constant of furosemide (30$\mu{M}$) were significantly (p<0.05) decreased by 30$\mu{M}$ of cefalexin, PAH, ibuprofen and PAS in rat small intestine. The plasma concentration of furosemide was significantly (p<0.01) increased by cefalexin, PAH and ibuprofen in rabbits. But the urinary excretion rate, renal clearance and diuretic action of furosemide were significantly (p<0.05) decreased by cefalexin, PAH, ibuprofen and PAS in rabbits.

  • PDF

Renal replacement therapy in neonates with an inborn error of metabolism

  • Cho, Heeyeon
    • Clinical and Experimental Pediatrics
    • /
    • 제62권2호
    • /
    • pp.43-47
    • /
    • 2019
  • Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonates.

타우린의 일반적 특성에 관한 선행연구 고찰 (General Characteristics of Taurine: A Review)

  • 윤진아;최경순;신경옥
    • 한국식품영양학회지
    • /
    • 제28권3호
    • /
    • pp.404-414
    • /
    • 2015
  • Taurine is one of the most abundant free ${\beta}$-amino acids in the human body that accounts for 0.1% of the human body weight. It has a sulfonic acid group in place of the more common carboxylic acid group. Mollusks and meat are the major dietary source of taurine, and mother's milks also include high levels of this amino acid. The leukocytes, heart, muscle, retina, kidney, bone, and brain contain more taurine than other organs. Furthermore, taurine can be synthesized in the brain and liver from cysteine. There are no side effects of excessive taurine intake in humans; however, in case of taurine deficiency, retinal abnormalities, reduced plasma taurine concentration, and other abnormalities may occur. Taurine enters the cell via a cell membrane receptor. It is excreted in the urine (approximately 95%) and feces (approximately 5%). Taurine has a number of features and functions, including conjugation with bile acid, reduction of blood cholesterol and triglyceride levels, promotion of neuron cell differentiation and growth, antioxidant effects, maintenance of cell membrane stability, retinal development, energy generation, depressant effects, regulation of calcium level, muscle contraction and relaxation, bone formation, anti-inflammatory effects, anti-cancer and anti-atherogenic effects, and osmotic pressure control. However, the properties, functions, and effects of taurine require further studies in future.

Antidiabetic and Hypolipidemic Effects of Collybia confluens Mycelia Produced by Submerged Culture in Streptozotocin-Diabetic Rats

  • Yang, Byung-Keun;Jeong, Sang-Chul;Lee, Hyun-Ji;Sohn, Dong-Hwan;Song, Chi-Hyun
    • Archives of Pharmacal Research
    • /
    • 제29권1호
    • /
    • pp.73-79
    • /
    • 2006
  • This investigation was undertaken to study the effects of oral administration (3 weeks) of Collybia confluens mycelial powder (CCMP) produced by a submerged culture on plasma glucose and other biochemical parameters in streptozotocin (STZ)-induced diabetic rats. Antidiabetic and hypolipidemic effects were proportionally increased with the increasing concentration of the CCMP for oral administration. The CCMP, at the dose of 400 mg/kg BW, substantially reduced the plasma glucose level by as much as 33.1 % as compared to the STZ-induced diabetic rats group. It also lowered the plasma total cholesterol, triglyceride, and low density lipoprotein (LDL) cholesterol by 22.9%, 19.9%, and 37.3%, respectively. The levels of total cholesterol and triglyceride in liver were reduced to the extent of by 13.5% and 18.8%, and the activity of alanine transaminase (ALT) and aspartate transaminase (AST) was decreased by 48.8% and 37.2%, respectively, under the influence of CCMP. The general components of CCMP were found to contain 26.18% carbohydrate, 3.67% crude ash, 4.02% crude fat, 22.55% crude protein, and 43.58% dietary fiber. The amino acid composition of the CCMP was also analyzed in detail.

Effects of Dietary Supplementation with Branched-chain Amino Acids (BCAAs) during Nursing on Plasma BCAA Levels and Subsequent Growth in Cattle

  • Li, J.Y.;Suzuki, K.;Koike, Y.;Chen, D.S.;Yonezawa, T.;Nishihara, M.;Manabe, N.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • 제18권10호
    • /
    • pp.1440-1444
    • /
    • 2005
  • To determine the effects of short-term dietary supplementation of branched-chain amino acids (BCAAs) during nursing (from 3 to 28 days of age) on plasma BCAA levels and subsequent growths in cattle, 12 nursing male Holstein calves, randomly assigned to control and treatment groups (n = 6 in each group), orally received a daily supplement of essential BCAAs (2 g/kg body weight/day; 1:1:1 of valine, leucine and isoleucine) or not. The plasma BCAA levels increased linearly after the administration. During the treatment period, average daily gain (ADG) was lower in the treatment group (0.43${\pm}$0.07 kg/day) than the controls (0.71${\pm}$0.07 kg/day, p<0.05). However, at 2 months of age, ADG was significantly higher in the BCAA-treated group (1.16${\pm}$0.26 kg/day vs. 0.51${\pm}$0.06 kg/day, p<0.05). Furthermore, at age 8, 9 and 10 month, ADG in the treated group (1.35${\pm}$0.23, 1.46${\pm}$0.07 and 1.60${\pm}$0.16 kg/day, respectively) showed a linear increase and was significantly higher than that in the control group (0.88${\pm}$0.14, 0.70${\pm}$0.21 and 1.11${\pm}$0.11 kg/kg, respectively, p<0.05). Overall, ADG was 15.6% higher in the treatment group (1.26${\pm}$0.05 kg vs. 1.09${\pm}$0.04 kg; p<0.05). The final body weight at slaughter was 14.8% higher in the treatment group (759.5${\pm}$17.7 kg vs. 661.7${\pm}$21.2 kg, p<0.01). Thus, the supplementation of BCAAs during nursing improves ADG and carcass weight in cattle and is a useful husbandry technique for beef cattle.

Saccharomyces cerevisiae에서 Neurospora crassa coq-4 유전자의 기능적 발현 (Functional Expression of the Neurospora crassa coq-4 Gene in Saccharomyces cerevisiae.)

  • 김은정;최상기;천재우;오계헌;이병욱
    • 미생물학회지
    • /
    • 제40권2호
    • /
    • pp.73-80
    • /
    • 2004
  • Coenzyme Q는 지용성 퀴논 유도체로서 미토콘드리아의 내막과 원핵생명체의 세포막에 위치하는 전자전달계에서 전자 운반체로 이용되며 또한 항산화제의 기능도 갖는다. Coenzyme Q의 생합성에 관여하는 Saccharomyces cerevisiae의 coq4 유전자에 유사성을 나타내는 Neurospora crassa coq-4유전자를 클로닝하여 S. cerevisiae coq4 돌연변이체에서 기능적으로 발현하였다. 상보된 S. cerevisiae 균주들은 coenzyme $Q_{6}$의 생산능력을 회복하였으며 정상적인 성장률을 보였다. 또한 linoleic acid 및 linolenic acid와 같은 불포화지방산에 대한 낮은 감수성을 보였다. N. crassa의 COQ4 단백질은 39.7 kDa의 분자량을 갖는 347개의 아미노산으로 구성되어 있는 것으로 예상되며, S. cerevisiae의 Coq4p와 35%의 일치도 및 52%의 유사도를 보인다.

율무쌀의 경구투여(經口投與)가 백서(白鼠)의 성장(成長)에 미치는 영향(影響) (Effects of Pearl Barley on the Growth and Development of Albino Rats)

  • 조수열;박명희
    • 한국식품영양과학회지
    • /
    • 제6권1호
    • /
    • pp.41-47
    • /
    • 1977
  • 율무쌀의 영양적(營養的)인 가치(價値)를 평가(評價)하기 위하여 체중(體重) $55.6{\pm}3.5g$의 Leuise계(系) albino rats 암수 각(各) 40수(首)를 8 group으로 나누고 각(各) group에 casein 15%+starch 75%+oil 8.0% diet(A), 율무쌀 98% diet(B), 쌀 98% diet(C), 보리쌀 98% diet(D), 쌀 49%+율무쌀 49% 혼합 diet(E), 쌀 73.5%+율무쌀 24.5% 혼합 diet(F), 보리쌀 49%+율무쌀 49% 혼합 diet(G), 보리쌀 73.5%+율무쌀 24.5% 혼합 diet(H)의 8종류(種類)의 diet에 각각 비타민 mixture 1% 및 salt mixture 1% 씩을 첨가(添加)한 것을 7주간(週間) 급여(給與)하여 체중증가량(體重增加量), 소화율(消化率), liver의 중량(重量), liver 및 plasma중의 protein 양(量), plasma 중의 free amino acid양(量), hematocrit 및 hemoglobin양(量)을 측정(測定)한 바 다음과 같은 결과(結果)를 얻었다. 1. 체중증가량(體重增加量)은 율무쌀 98% diet(B) group 이 가장 낮았고, 다음이 쌀 49%+율무쌀49% 혼합 diet(E) group으로써 이들 사이에는 유의(有意)한 차(差)가 없었으나 쌀 98% diet(C) group, 보리쌀 98% diet(D) group 및 기타 혼식(混食) group과는 유의(有意)한 차(差)(P<0.01)가 있었다. 2. 율무 현미(玄米) 및 백미(白米)의 소화율(消化率)은 protein과 carbohydrate는 백미(白米)가 높았고 fat는 현미(玄米)가 높았다. 3. liver의 중량(重量)은 율무쌀 98% diet(B) group은 쌀 98% diet(C) group과는 유의(有意)한 차(差)(P<0.05)가 있었으나 기타 group과는 유의(有意)한 차(差)가 없었다. 그리고 단위체중(單位體重)에 대한 무게는 차이(差異)가 없었다. 4. live 및 plasma의 total protein과 plasma의 A/G ratio는 율무쌀 98% diet(B) group이 가장 낮았다. 5. plasma 중의 free amino acid 조성(組成)은 식이(食餌)의 조성(組成)과 비슷하였다. 6. hematocrit와 hemoglobin content는 율무쌀 98% diet(B) group이 가장 낮았다. 7. 혼식(混食) group에 있어서는 쌀과 율무쌀 및 보리쌀과 율무쌀의 혼합비율(混合比率)이 1:1보다 3:1이 더 좋은 결과(結果)이었다.

  • PDF