• Title/Summary/Keyword: Protein Deficiency

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A Literature Study of The Osteomalacia (골연화증(骨軟化症)에 대(對)한 동서의학적(東西醫學的) 문헌고찰(文獻考察))

  • Park, Jong-Hyuck;Hwang, Young-Geun;Jeong, Ji-Gheon
    • The Journal of Dong Guk Oriental Medicine
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    • v.8 no.1
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    • pp.159-169
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    • 1999
  • Osteomalacia is syndrome of diverse etiology. characterized pathophysiologically by a failure of normal mineralization of bone and epiphyseal cartilage. This study was performed to investigate causes of disease, pathogenic mechanisms, symptoms, therapies and precriptions through the successive medical literatures. recent chinese medical literatures and chinese medical journals. It is similar to atrophic debility of bones, bone leaning, bone exhaustion, rheumatism involving the bone, osteodynia and cold and heat of bone etc. of oriental medicine. The most principal cause of this is deficiency of kidney. similar to hypophosphatemia caused by increased renal clearance and deficiency of vitamin D, and the rest are senility, deficiency of spleen, deficiency of qi and deficiency of blood. There are nourishing the kidney and spleen, nourishing the qi and blood, warming and passing the muscle and mac, passing an articulation an invigorating the muscle and bone, in principal therapy. And in medical herbs are rehmanniae radix preparat, corni fructus, discoreae rhizoma, cuscutae semen, tigridis os, juglandis semen, hominis placenta, drynariae rhizoma, eucommiae cortex, cynomorii herba, cervi cornus colla, cervi pantotrichum cornu, moutan cortex, polygoni multiflori radix, angelicae gigantis radix, achyranthis bidentatae radix, cibotii rhizoma, hirudo, eupolyphaga, spatholobi caulis, salviae miltiorrhizae radix, draconis resina, curcumae longae rhizoma. In care there are a sun-bath, exercise, high protein diet and taking vitamin D. And they reduce smoking, coffee, drinking etc.

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A Case of Ornithine Transcarbamylase Deficiency in a Boy with Neonatal Seizure and Altered Mentality (신생아기 경련 및 의식저하를 주소로 내원한 Ornithine Transcarbamylase Deficiency 남아 1례)

  • Im, Minji;Song, Ari;Lee, Soo-Youn;Park, Hyung-Doo;Cho, Sung Yoon;Jin, Dong-kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.2
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    • pp.55-61
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    • 2018
  • Orinithine transcarbamylase (OTC) deficiency is the most common inborn error of the urea cycle with resulting hyperammonemia, which is medical emergency in newborns.We recently had a case of a boy that presented with lethargy, seizure, hyperammonemia and hypocalcemia in neonatal period. He was diagnosed with OTC deficiency by two consequent ways which are initial biochemical phenotype including hyperammonemia and an increased orotic acid in his urine and genetic analysis of the OTC gene. The OTC gene showed a novel hemizygous mutation c.913C>T (p.Pro305Ser). He was treated by low protein intake, sodium benzoate, phenylbutyrate sodium, L-arginine, and continuous renal replacement therapy (CRRT). After discharge, he has a relatively good prognosis without notable developmental delay. For good prognosis, the duration of hyperammonemia should be shorten. And it can be reached by an early diagnosis. For early detection of OTC deficiency, targeted exome sequencing will be a important role as well as biochemical tests.

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Floating Thrombus in Aortic Arch

  • Noh, Tae Ook;Seo, Pil Won
    • Journal of Chest Surgery
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    • v.46 no.6
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    • pp.464-466
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    • 2013
  • Floating thrombi in the aortic arch are very rare and an unusual source of systemic embolism. Herein, a case of a 3-cm thrombus in the aortic arch is reported. It was a floating, highly mobile thrombus attached to the lesser curvature of the aortic arch. The patients had a hypercoagulable disorder induced by protein C and S deficiency. The thrombus was operatively removed with a favorable outcome.

Effect of Genistein and Soy Protein on Lipids Metabolism in Ovariectomized Rats (난소절제 랫드에서 제니스테인과 콩단백질이 지질 대사에 미치는 영향)

  • Lee Young Min;Jung Myeong Ho;Lee Yeon Sook;Song Jihyun
    • Journal of Nutrition and Health
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    • v.38 no.4
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    • pp.267-278
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    • 2005
  • Postmenopausal women or ovariectomized rats are associated with increased cholesterol levels, which are risk factors of metabolic syndrome and cardiovascular diseases. Increased prevalence of metabolic syndrome after menopause might be associated with estradiol deficiency. Harmful effect of estradiol hampers the casual usage of hormone to prevent the metabolic syndrome. Soy protein has been reported to show several beneficial effects on health, however it is unclear which components of soy protein is responsible for anti-obesity and hypocholesterolemic effects. Soy isoflavones, gem-stein and daizein, are suggested to have anti-obesity and hypocholesterolemic effects but with inconsistency. The present study investigated the effect of supplementation of genistein (experiment I) and soy protein containing isoflavones (experiment II) to high fat diet on body weight gain, food intake, liver and fat tissue weight and the lipid levels in ovariectomized rats. Plasma and hepatic lipid contents and the mRNA levels of genes encoding lipid metabolism related proteins, such as CPT1 and HMGR were measured. Ovariectomy increased body weight, fat tissue weight and plasma and hepatic lipid levels which increase the risk of metabolic syndrome. Soy protein could improve plasma and hepatic lipids levels. Soy protein also increased hepatic CPT1 and HMGR mRNA levels. Plasma and hepatic lipids levels could not be decreased by dietary genistein alone. In contrast, lipids levels could be decreased by isoflavone-fortified soy protein, suggesting that the ingestion of soy protein enriched with isoflavone gives more benefit for protecting postmenopausal women from metabolic syndrome.

Effect of Dietary Protein and Calcium Levels on Calcium Metabolism of the Rat (식이단백질과 칼슘 수준이 흰쥐의 칼슘대사에 미치는 영향)

  • Yoon, Gun-Ae;Hwang, Hye-Jin
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.34 no.2
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    • pp.176-180
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    • 2005
  • This study was conducted to examine effect dietary protein and calcium levels on calcium metabolism of the rat. Weaned 6-week old male rats were divided into 4 groups and were fed experimental diets for six weeks. Experimental groups were HPNC group-high protein normal calcium (protein: 400 g/kg diet, calcium: 0.5%), HPLC group-high protein low calcium (protein: 400 g/kg diet, calcium: 0.1%), NPNC group-normal protein normal calcium (protein: 200 g/diet, calcium: 0.5%), NPLC group-normal protein low calcium (protein: 200 g/diet, calcium: 0.1%). The calcium excretion in urine was higher in high protein group than in normal protein group, and it was highest in HPLC group. The activation of alkaline phophatase had a tendency to low in normal calcium group, and the concentration of parathyroid hormone (PTH) was the lowest in HPLC group. The deoxypyridinoline (DPD) concentration of urine was investigated as the highest in HPLC group and it was significantly lower in HPNC group that consumed normal calcium. The bone density of the femur was the highest in NPNC group and the lowest in NPLC group. As the results of this study, calcium excretion in urine and DPD density were the highest and the bone density was the lowest in HPLC group. It may suggest that the deficiency of calcium causes adversely effect in calcium metabolism upon consuming high protein diet. Therefore, it should be emphasized to consume enough calcium to prevent the hindrance of skeletal metabolism caused by deficiency of calcium upon consuming high protein diet.

Membrane associated Ca2+ buffers in the heart

  • Lee, Duk-Gyu;Michalak, Marek
    • BMB Reports
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    • v.43 no.3
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    • pp.151-157
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    • 2010
  • $Ca^{2+}$ is a universal signalling molecule that affects a variety of cellular processes including cardiac development. The majority of intracellular $Ca^{2+}$ is stored in the endoplasmic and sarcoplasmic reticulum of muscle and non-muscle cells. Calreticulin is a well studied $Ca^{2+}$-buffering protein in the endoplasmic reticulum, and calreticulin deficiency is embryonic lethal due to impaired cardiac development. Despite calsequestrin being the most abundant $Ca^{2+}$-buffering protein in the sarcoplasmic reticulum, viability is maintained in embryos without calsequestrin and normal $Ca^{2+}$ release and contractile function is observed. The $Ca^{2+}$ homeostasis regulated by the endoplasmic and sarcoplasmic reticulum is critical for the development and proper function of the heart.

Characterization of a gene encoding ornithine carbamoyltransferase from rice

  • Islam Sikdar, Shafiqul;Kim, Jung-Sup
    • Journal of Plant Biotechnology
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    • v.36 no.4
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    • pp.397-402
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    • 2009
  • Ornithinine carbamoyltransferase (OTC) is an enzyme that catalyzes the key step in arginine biosynthesis in bacteria and plants. OTC is also involved in the urea cycle and deficiency of the enzyme in human leads to disease. The argF gene encoding OTC has been reported in many bacteria and few plants. Here we report the characterization of a gene encoding OTC from rice (OsOTC). Analysis of a cDNA sequence from rice revealed that the full-length open reading frame of OsOTC consisted of 367 amino acids, corresponding to a protein of approximately 39.7 kDa. The predicted amino acid sequence of OsOTC harbor distinct five OTC signature sites and is highly homologous to that of enzymes of plants, animals and many bacterial OTCs. Expression of OsOTC in argF mutants of Escherichia coli showed that the gene was able to functionally complement to the mutant. These results suggest that the OsOTC encode a protein for ornithine carbamoyltransferase in rice.

The First Case of Korean Boy with Mitochondrial Trifunctional Protein Deficiency Diagnosed by Acylcarnitine Profiles and DNA analysis : A Novel Mutation in the α-subunit of the Mitochondrial Trifunctional Protein and a Unusual Intergenic Sequence with Two Polymorphisms

  • Lee, Ji-Eun;Yoon, Hye-Ran;Paik, Kyung Hoon;Hwang, Jong Hee;Hwang, Soo-Jung;Shim, Jae-Won;Chang, Yun-Sil;Park, Won-Soon;Strauss, Arnold W.;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.2 no.1
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    • pp.12-14
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    • 2002
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A proteomic approach to identify of yeast proteins that related with accumulation of misfolded protein in cell

  • Shin, Yong-Seung;Seo, Eun-Joo;Kim, Joon;Yu, Myeong-Hee
    • Proceedings of the Korean Biophysical Society Conference
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    • 2003.06a
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    • pp.64-64
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    • 2003
  • In growing number of diseases it has been shown that the aggregation of specific proteins has an important role in the pathogenesis of the disorder. This has been demonstrated in structural detail with the liver cirrhosis of ${\alpha}$$_1$-antitrypsin deficiency, and it is now believed that similar protein aggregation underlies many neurodegenerative disorders such as autosomal dominant Parkinson disease, prion diseases, Alzheimer disease, Huntington disease.

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Neuronal Autophagy: Characteristic Features and Roles in Neuronal Pathophysiology

  • Valencia, McNeil;Kim, Sung Rae;Jang, Yeseul;Lee, Sung Hoon
    • Biomolecules & Therapeutics
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    • v.29 no.6
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    • pp.605-614
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    • 2021
  • Autophagy is an important degradative pathway that eliminates misfolded proteins and damaged organelles from cells. Autophagy is crucial for neuronal homeostasis and function. A lack of or deficiency in autophagy leads to the accumulation of protein aggregates, which are associated with several neurodegenerative diseases. Compared with non-neuronal cells, neurons exhibit rapid autophagic flux because damaged organelles or protein aggregates cannot be diluted in post-mitotic cells; because of this, these cells exhibit characteristic features of autophagy, such as compartment-specific autophagy, which depends on polarized structures and rapid autophagy flux. In addition, neurons exhibit compartment-specific autophagy, which depends on polarized structures. Neuronal autophagy may have additional physiological roles other than amino acid recycling. In this review, we focus on the characteristics and regulatory factors of neuronal autophagy. We also describe intracellular selective autophagy in neurons and its association with neurodegenerative diseases.