• Title, Summary, Keyword: Protein Deficiency

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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
    • Yeungnam University Journal of Medicine
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    • v.31 no.1
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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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    • v.18 no.9
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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.

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

  • 조윤옥;박경순
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.24 no.4
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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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    • v.8 no.1
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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.

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

  • Nam, Yoon-Sung;Kim, Nam-Keun;Kang, Myung-Seo;Oh, Do-Yeon;Cha, Kwang-Yul
    • Clinical and Experimental Reproductive Medicine
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    • v.28 no.2
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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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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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    • v.1 no.1
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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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Different Level of Plasma Free Hemoglobin between Qi-deficiency and Fire Heat among Korean Stoke Subjects (한국인 중풍환자의 기허군 화열군의 plasma free hemoglobin의 비교)

  • Lim, Ji-Hye;Ko, Mi-Mi;Lee, Jung-Sup;Lee, Myeong-Soo;Cha, Min-Ho
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.25 no.4
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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.

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

  • Yoon, Mi;Kim, Seung-Oh;Kim, Jong-Soo;Yoo, Seung-Hoon
    • THE JOURNAL OF THE KOREAN ACADEMY OF PEDTATRIC DENTISTRY
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    • v.37 no.2
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    • pp.207-212
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    • 2010
  • Protein C deficiency increases the risk of thrombosis due to the lack of anticoagulant factor protein C. Among the numerous congenital protein C deficiencies, homozygous protein C deficiency has an especially low protein C activity level, that it is almost undetectable. It is a rare disease with a probability of 1:250000~500000. The signs and symptoms of homozygous protein C deficiency include purpuric, necrotic dermatosis, ecchymosis, blindness, and thrombosis in central nervous system. A 4-year-old girl was brought to the clinic with a chief complaint of extensive caries. The child was under warfarin medication in order to prevent possible complications during dental treatment. We consulted the pediatric department. Without warfarin intake, serious complications may occur due to thrombosis during dental treatment. Therefore, certain warfarin dosage (INR 3~5) and fresh frozen plasma as a backup for excessive hemorrhage were recommended. This child was a severely disabled child with the loss of vision, and it was difficult to manage her behavior effectively. Thus, dental treatment was carried out under general anesthesia, where bleeding control would be also easier to achieve.This report presents the case of a 4-year-old girl with protein C deficiency, who has received dental treatment for extensive caries under general anesthesia.

A Case of Pediatric Unprovoked Deep Vein Thrombosis due to Combined Hereditary Thrombophilia of Antithrombin III and Protein S Deficiency (유전성 항트롬빈과 S단백 결핍증으로 인한 소아 심부정맥혈전증 증례)

  • Kim, Jeong Yeon;Kang, I-Seok;Kim, Hee-Jin
    • Clinical Pediatric Hematology-Oncology
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    • v.25 no.2
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    • pp.185-190
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    • 2018
  • Unprovoked deep vein thrombosis (DVT) is uncommon in pediatric patients and, among those, combined hereditary thrombophilia is particularly rare. We present a 9-year-old Korean boy who developed lower extremity pain with swelling, and was diagnosed with unprovoked DVT due to hereditary (combined hereditary thrombophilia). Coagulation test revealed antithrombin III and protein S deficiency. The genetic work up confirmed the first case of combined antithrombin III deficiency and protein S deficiency by SERPINC1 heterozygous termination mutation [c.685C>T ($p.Arg229^*$)] and PROS1 heterozygous missense mutation [c.1597G>A (p.Val533Met)]. He was treated with continuous heparin and catheter intervention but those were ineffective or transiently effective. His DVT gradually improved only after prolonged anticoagulation.

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

  • 주윤옥
    • Journal of Nutrition and Health
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    • v.27 no.3
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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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