• 제목/요약/키워드: Protein Deficiency

검색결과 374건 처리시간 0.035초

Hereditary protein S deficiency presenting acute pulmonary embolism

  • Kim, Jiwan;Kim, Sung Hea;Jung, Sang Man;Park, Sooyoun;Yu, HyungMin;An, Sanghee;Kang, Seonghui;Kim, Hyun-Joong
    • Journal of Yeungnam Medical Science
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    • 제31권1호
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    • pp.52-55
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    • 2014
  • Protein S deficiency is one of the several risk factors for thrombophilia and can cause blood clotting disorders such as deep vein thrombosis and pulmonary embolism. A 54-year-old man was admitted with the complaint of dyspnea and was diagnosed with pulmonary embolism. The patient had very low level of free protein S, total protein S antigen, and protein S activity (type I protein S deficiency). In history taking, we found that his mother, 78 year old, had a history of same disease 10 years ago, and confirmed the pronounced low level of protein S. The patient's son also had very low level of protein S, however there had not been any history of pulmonary embolism yet. This case study suggests that asymptomatic persons with a family history of protein S deficiency and pulmonary embolism should be checked regularly for early detection of the disease, as protein S deficiency can be suspected.

The Effects of Dietary Lysine Deficiency on Muscle Protein Turnover in Postweanling Pigs

  • Chang, Yi-Ming;Wei, Hen-Wei
    • Asian-Australasian Journal of Animal Sciences
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    • 제18권9호
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    • pp.1326-1335
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    • 2005
  • The main purpose of this study is to investigate the effects of dietary lysine deficiency on protein turnover of porcine muscles. There were 18 LYD three-breed-crossing postweanling barrows from six litters cannulated with gastric tubes through the esophagus at approximate 10 kg of body weight and allocated into three treatment groups. When their body weights reached over 12 kg, one group was sacrificed for determining the initial protein masses of m. masseter, m. longissimus dorsi, m. adductor and m. biceps femoris from the right body side. The others received a diet containing 100% or 61.4% (calculated values) of the lysine requirement (NRC, 1998) multiplied by 1.103 for a period of 17 days. Daily feed provision was computed for each pig according to body weight at the same day. All pigs were infused a flooding dose of $^2$H$_5$-phenylalanine to determine the fractional protein synthesis rates (FSR) of the aforementioned muscles in the end. Their four muscles from the right body side were also dissected for measuring the fractional rates of protein accretion (FAR). As for protein degradation, fractional rates (FDR) were calculated by differences between synthesis and accretion. Results showed that the lysine deficiency resulted in, significantly (p<0.05), lighter body weights, smaller muscles and a slower growth rate. The protein mass, accreted by the muscles, of the deficient group was only 54% averaged of the pigs fed adequately (p<0.05). The FAR of these muscles in the deficient group was significantly lower (p<0.05) and only achieved 61.1% averaged of the control; there was no significant difference (p>0.05), nevertheless, in the amino-acid composition of muscles between two groups. The lysine deficiency reduced significantly (p<0.05) the FSR of m. longissimus dorsi but did not influence its FDR. The m. biceps femoris also presented an inhibited FSR while its FDR reduced only exhibited a very high tendency (p = 0.055) compared to the adequately-fed pigs. As for the m. masseter and m. adductor, both of the FSR and FDR were depressed significantly (p<0.05) by the lysine deficiency, and changes in the FSR were severer than those in the FDR, so that their FAR were significantly slower (p<0.05) in comparison with the control group. The lysine deficiency also inhibited the RNA translation activity of the muscles while the effects on RNA capacity were not significant (p>0.05). In conclusion, the FAR of muscle protein was changed by the current lysine deficiency through the alterations in the FSR and/or FDR.

Vitamin $B_2$ 결핍이 Streptozotocin 유발 당뇨 흰쥐의 에너지대사에 미치는 영향 (The Effect of Vitamin $B_2$ Deficiency on Fuel Metabolism in Streptozotocin Induced Diabetic Rats)

  • 조윤옥;박경순
    • 한국식품영양과학회지
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    • 제24권4호
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    • pp.487-492
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    • 1995
  • The purpose of this study was to investigate the effect of vitamin B2 deficiency on fuel metabolism in streptozotocin-induced diabetic rats. Thirty rats were fed a vitamin B2 deticient diet(-B2) or a control diet (+B2) for 2 weeks and then subdivided into 3 groups respectively : base group, one day diabetic group and three day diabetic group. Diabetes of the rats were induced by streptozotocin injection into the tail vein. Glucose, glycogen, protein, alanine, triglyceride and free fatty acid were compared in plasma, liver, skeletal muscle of rats. Also, the total urinary nitrogen and glucose excertion were compared. Compared with +B2 rats, the increase of plasm glucose in -B2 rats due to the diabetes tended to be smaller. After diabetes were induced, the levels of plasma protein and alanine was significantly decreased and the urinary nitrogen excretion was significantly increased in -B2 rats. The level of plasma free fatty acid was increased continuously in B2 rats while increased at the first day and decreased at the third day diabetes was induced in +B2 rats. These results suggest that vitamin B2 deficiency increase protein catabolism due to the decrease of fatty acid oxidation. Thus, vitamin B2 deficiency in diabetes impair the adaptation of animals to the fuel metabolism and aggravate the body protein wasting which is one of the chronic complications of diabetes.

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Effects of Sulfur Fertilizer on the Expression of 11S and 7S Seed Storage Proteins of Soybean

  • El-Shemy Hany A.;Nguyen Nguyen Tran;Ahmed Sherif H.;Fujita Kounosuke
    • Journal of Plant Biotechnology
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    • 제8권1호
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    • pp.1-8
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    • 2006
  • The differential response of soybean cultivars with or without sulfur (S) application was observed under fold conditions. Plant biomass decreased by sulfur deficiency but the reduction was less in Bragg variety about 26 % relative to the control than other ones over 45%, probably due to less reduction in loaves and pods. The photosynthetic rate of Bragg cultivar was also unaffected by the absence of sulfur application while it depressed in other lines. Soybean cultivars were compared in terms of storage protein, protein quality and biomass production by application of sulfur nutrition. The storage protein concentration tended to decrease without sulfur application in all the cultivars, however the differential response of protein quality only by 11S/7S ratio to sulfur nutrition status was observed: For instance, Bragg cultivar had higher biomass and protein production but protein quality decreased at sulfur deficiency. On the other hand, biomass and protein production in other cultivars remained louver at sulfur deficiency but protein quality differed genetically in spite of sulfur nutrition status. These results suggest that the response of soybean to sulfur nutrition is controlled by genotypic difference and sulfur supply status.

항인지질증후군에서 S단백질 결핍증에 대한 연구 (A Study of Protein S Deficiency in Antiphospholipid Syndrome)

  • 남윤성;김남근;강명서;오도연;차광열
    • Clinical and Experimental Reproductive Medicine
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    • 제28권2호
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    • pp.105-110
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    • 2001
  • Objective: To evaluate the abnormality of protein S in patients with recurrent spontaneous abortion due to antiphospholipid syndrome. Material and Method: Antigen and activity of protein S were analyzed by enzyme immunoassay and clotting method, respectively. Results: Of 18 patients with antiphospholipid syndrome, 4 patients were found to have no abnormality of protein S. There were 14 cases of protein S abnormality. Among them, there were 8 cases of type 1, 1 case of type 2, and 5 cases of type 3 protein S deficiency. Conclusion: So in the workup of patients with recurrent spontaneous abortion due to antiphospholipid syndrome, the evaluation for protein S is required.

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ANKS1A-Deficiency Aberrantly Increases the Entry of the Protein Transport Machinery into the Ependymal Cilia

  • Haeryung Lee;Jiyeon Lee;Miram Shin;Soochul Park
    • Molecules and Cells
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    • 제46권12호
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    • pp.757-763
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    • 2023
  • In this study, we examine whether a change in the protein levels for FOP in Ankyrin repeat and SAM domain-containing protein 1A (ANKS1A)-deficient ependymal cells affects the intraflagellar transport (IFT) protein transport system in the multicilia. Three distinct abnormalities are observed in the multicilia of ANKS1A-deficient ependymal cells. First, there were a greater number of IFT88-positive trains along the cilia from ANKS1A deficiency. The results are similar to each isolated cilium as well. Second, each isolated cilium contains a significant increase in the number of extracellular vesicles (ECVs) due to the lack of ANKS1A. Third, Van Gogh-like 2 (Vangl2), a ciliary membrane protein, is abundantly detected along the cilia and in the ECVs attached to them for ANKS1A-deficient cells. We also use primary ependymal culture systems to obtain the ECVs released from the multicilia. Consequently, we find that ECVs from ANKS1A-deficient cells contain more IFT machinery and Vangl2. These results indicate that ANKS1A deficiency increases the entry of the protein transport machinery into the multicilia and as a result of these abnormal protein transports, excessive ECVs form along the cilia. We conclude that ependymal cells make use of the ECV-based disposal system in order to eliminate excessively transported proteins from basal bodies.

Effects of an Anabolic Steroid, Nandrolone Phenylpropionate, on Reductions in Body and Muscle Proteins Under the Dietary Regimens of Feeding a Low-Protein Diet and of 50% Food Restriction in Rats

  • Choo, Jong-Jae
    • Nutritional Sciences
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    • 제1권1호
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    • pp.3-7
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    • 1998
  • The aim of the present investigation was to see whether an anabolic steroid, nandrolone phenylpropionate (NPP), exerts protienanabolic effects under such adverse nutritional conditions as protein deficiency and protein-energy malnutrition in male rats. feeding on a low-protein (8% casein) diet resulted in a marked reduction in body weight gain that was associated with reductions in body protein and protein content of gastrocnemius muscle. Administration of NPP (4 mg/kg body weight) did not alter muscle and body protein depletion induced by a low-protein diet. 50% food restriction caused reductions in body protein and in protein content of gastrocnemius muscle. These reductions were partially prevented by NPP (4 mg/kg body weight). Food restriction did not affect plasma concentration of corticosterone, insulin, or tetosterone plus dihydrotestosterone. On the other hand, neither plasma concentration of corticosterone nor insulin were affected by NPP. The present results show that anabolic steroids do not express anabolic effects under conditions of protein deficiency, but in protein-energy malnutrition, anabolic steroids exert their anabolic effects even in male rats.

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한국인 중풍환자의 기허군 화열군의 plasma free hemoglobin의 비교 (Different Level of Plasma Free Hemoglobin between Qi-deficiency and Fire Heat among Korean Stoke Subjects)

  • 임지혜;고미미;이정섭;이명수;차민호
    • 동의생리병리학회지
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    • 제25권4호
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    • pp.697-701
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    • 2011
  • The purpose of this study was to fine proteins, which have significantly different level in plasma between Qi-deficiency and Fire-heat group of Korean Oriental Stroke pattern identification (PI) among Korean stroke patients. Eighteen stroke patients with Qi-deficiency and forty nine patients with Fire-heat, which had critical syndrome of each PI, were participated in this study. Plasma protein pattern were analyzed by SELDI-TOF MS using Q10 strong anion exchange chip and Mass spectral data (m/z) statistically determined. The expression level of proteins, which were different between Qi-deficiency and Fire-heat in the results by SELDI-TOF MS, were confirmed by western blot. As a result of analyzing plasma protein by SELDI-TOF MS, six protein peaks were significantly higher in Fire-heat group than Qi-deficiency group. Two peaks among of them, M15003 and M15745, were respectively identified as hemoglobin alpha and beta in previous study. Expression level of plasma free hemoglobin of Fire-heat group was also confirmed higher in Fire-heat group than in Qi-deficiency group. These findings suggest that plasma free hemoglobin is a candidate for discriminating Qi-deficiency and Fire-heat group according to pattern identification (PI) of stroke.

동형접합성 단백 C 결핍 환아의 치과적 치험례 (DENTAL TREATMENT OF A PEDIATRIC PATIENT WITH HOMOZYGOUS PROTEIN C DEFICIENCY: A CASE REPORT)

  • 윤미;김승오;김종수;유승훈
    • 대한소아치과학회지
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    • 제37권2호
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    • pp.207-212
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    • 2010
  • 단백 C 결핍증은 항응고인자인 단백 C의 결핍으로 혈전 색전증의 위험성이 높다. 선천성 단백 C 결핍증 중 동형접합성 단백 C 결핍증은 단백 C의 활성도가 측정되지 않을 정도로 낮고 1/25만~50만의 발생 빈도를 가지는 희귀한 질환이다. 동형접합성 단백 C 결핍증의 주요 증상은 자반성 및 괴사성 피부 병변, 반상출혈, 실명, 중추신경계의 혈전증 등이다. 본 증례는 동형접합성 단백 C 결핍증인 만 4세 여아로 치아의 전반적인 우식을 주소로 내원하였다. 본 환아는 와파린 복용 중이었고 치과치료시 합병증의 예방을 위해 소아청소년과에 협진 의뢰하였다. 와파린 복용 중단 시 혈전으로 인한 심각한 합병증이 예상되어 와파린을 적절하게 (INR 3~5) 복용하되 치과 치료 중 과다 출혈 시 신선냉동혈장으로 조절할 것을 권고받았다. 이 환아는 실명을 동반한 중증 장애 아이로 행동조절이 어려웠고 과다 출혈시 신속한 처치가 가능하도록 하기 위해 전신마취 하에 치과치료를 시행하였다. 저자는 치아의 전반적인 우식을 주소로 내원한 만 4세 단백 C 결핍 환아에 대하여 전신마취 하 치과치료를 시행하였으며 다소의 지견을 얻었기에 보고하는 바이다.

Vitamin B6 결핍이 Streptozotocin 유발 당뇨 흰쥐의 에너지 대사물 농도에 미치는 영향 (The Effect of Vitamin B6 Deficiency on Energy Metabolite in Streptozotocin-induced Diabetic Rats)

  • 주윤옥
    • Journal of Nutrition and Health
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    • 제27권3호
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    • pp.228-235
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    • 1994
  • The purpose of this study was to investigate the effect of vitamin B6 deficiency on the concentration of energy metabolite in streptozotocin-induced diabetic rats. Thirty rats were fed a vitamin B6 deficient diet(-B6) or a control diet(+B6) for 5 weeks and then subdivided into 3 groups respectively ; base group, one day diabetic group and three day diabetic group. Diabetes of rats were induced by streptozotocin injection into the tail vein. Glucose, glycogen, protein, alanine, triglyceride and free fatty acids were compared in plasma, liver skeletal muscle of rats. Also, the total urinary nitrogen and glucose excretion were compared. Compared with +B6 rats, the increase of plasma glucose in -B6 rats due to the diabetes was smaller. After diabetes was induced, the level of plasma alamine was not changed in -B6 rats while increased significantly(p<0.05) in +B6 rats. The increase of urinary nitrogen excretion was smaller and the increase of muscle protein was larger in -B6 rats at the first day diabetes was induced. The levels of plasma free fatty acid and liver triglyceride were significantly (p<0.05) higher in -B6 rats after diabetes was induced. These results suggest that vitamin B6 deficiency may impair the adaptation of animals to the energy metabolism related due to a decrease of the body protein catabolism of fatty acid oxidation in diabetes and aggravate fatty liver which is one of the chronic complications of diabetes.

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