• 제목/요약/키워드: Protein C deficiency

검색결과 113건 처리시간 0.029초

만성폐쇄성폐질환을 동반한 광물성분진 노출 이직근로자의 철 결핍 (Iron deficiency in Retired Workers exposed to Mineral dust with Chronic Obstructive Pulmonary Disease)

  • 이종성;신재훈;백진이;정지영;김형근;최병순
    • 한국산업보건학회지
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    • 제29권1호
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    • pp.42-49
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    • 2019
  • Objective: Chronic obstructive pulmonary disease(COPD) is characterized by persistent airflow limitations associated with chronic inflammatory response due to noxious particles or gases in the lung. Iron deficiency is associated with chronic inflammation, such as COPD. The aim of this study was to evaluate the relationship among iron deficiency, iron homeostasis, and inflammation in retired miners with COPD. Methods: The serum levels of ferritin, soluble transferrin receptor(sTfR), and transferrin saturation(TSat) as biomarkers for iron deficiency and high-sensitivity C-reactive protein(hsCRP) as a biomarker for inflammation and hepcidin as a biomarker for iron homeostasis were measured in 93 male subjects. Iron deficiency was defined as any one or more of (1) sTfR>28.1 nmol/L, (2) TSat<16%, and (3) ferritin< $12{\mu}g/L$. Results: Iron deficiency was found 28% of the study subjects. Median levels of serum hsCRP was significantly increased related to airflow limitation of COPD(GOLD 1, $0.09{\mu}g/dL$ vs. GOLD 2, $0.17{\mu}g/dL$ vs. GOLD $3{\leq}$, $0.30{\mu}g/dL$, p=0.010), and was positively correlated with hepcidin(p=0.009). Mean level of serum hepcidin was lower in COPD subjects with iron deficiency(p=0.004) and serum levels of hepcidin was negatively correlated with %$FEV_1$ predicted(p=0.030). Conclusions: These results suggest that high serum levels of hepcidin are related to severe airflow limitation or inflammation and can decrease iron availability, regardless of iron status.

난소 과자극증후군과 동반된 뇌바닥동맥 혈전증 (A Case of Basilar Arterial Thrombosis in Ovarian Hyperstimulation Syndrome)

  • 박준철;임수연;배진곤;김종인;이정호
    • Clinical and Experimental Reproductive Medicine
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    • 제35권1호
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    • pp.83-88
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    • 2008
  • 보조생식술 시행 후 혈전색전증의 발생은 매우 드물기는 하나 치명적인 합병증이다. 과배란 유도시 중증 난소 과자극 증후군은 $0.1{\sim}0.5%$에서 발생하며, 중증 난소 과자극증후군 환자의 128명 중 한 명에서 혈전색전증이 발생하는 것으로 알려져 있다. 발병기전으로는 과배란 유도에 따른 고에스트로젠 혈증에 의한 혈액응고인자의 변화, 혈관 투과성의 증가에 따른 혈액농축 및 순환혈액의 감소 등을 원인으로 추정하고 있으나 그 정확한 기전은 알려진 바가 없다. 또한 thrombophilia나, 혈전색전증의 과거력 및 가족력이 있는 경우 발병율이 증가한다. 혈전증의 발생 부위는 정맥이 67%이나, 33%는 동맥에 발병하며 주로 뇌동맥에 발생된다고 보고되었다. 본 예는 3년간의 이차성 불임을 주소로 내원하여 시험관 아기시술을 시행하였으며 hCG 투여 8일 후 난소 과자극증후군이 중등도로 발생하였다. hCG 투여 후 11일째 갑작스런 의식 소실 및 우측 상지의 운동장애가 발생하여 시행한 MRI상 뇌바닥동맥 (basilar a.) 혈전증으로 진단되었으며, 혈관내 혈전 용해술 및 풍선 확장술 시행후 재관류에 성공하였다. 시술 2일 후 의식 및 운동장애는 완전히 회복되었으며 시술 7일 후 말더듬증 역시 회복되어 후유장애 없이 건강한 상태이다. Thrombophilic study상 Protein S결핍 소견을 보였고, 또한 vWF-associated Ag.이 증가된 양상을 보였다. 지금까지 국내에서 난소 과자극증후군 이후에 발생한 뇌경색은 5예가 보고되었으며, 이중 4예에서 protein S deficiency 소견을 보였으므로 선별 검사로 유용할 것으로 사료된다. 저자 등은 3년간의 이차성 불임을 주소로 내원한 33세 환자에서 시험관 아기시술시 hCG 투여 11일째에 발생한 뇌바닥동맥 혈전증을 간단한 문헌 고찰과 함께 보고하는 바이다.

A Korean Family with Cholesterol Ester Transfer Protein Deficiency

  • Youn, Seo-Young;Shin, Ik-Soon;Hong, Yong-Hee;Lee, Dong-Hwan
    • Journal of Genetic Medicine
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    • 제9권1호
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    • pp.38-41
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    • 2012
  • A 32-year-old female patient and her sister show high levels of high density lipoprotein (HDL) cholesterol in regular health checkups, since female patient was 11 years old. The patient's serum total cholesterol was 285 mg/dL and HDL cholesterol was 113 mg/dL. Her sister's total cholesterol was 240 mg/dL and the HDL cholesterol measured to be 90 mg/dL. Lipoprotein pattern and cholesteryl ester transfer activity gene analysis were examined in these patients. We found c.1321+1G>A (IVS14+1G/A) hetero mutation in cholesteryl ester transfer protein (CETP) genes. Generally, CETP mediates transfer and exchange of triglycerides and cholesteryl ester between plasma lipoproteins. Also we investigated a key role of HDL-CE and Apo A-1 metabolism. Patients with low levels of CETP have increased serum HDL levels. We hereby report two Korean cases of CETP deficiency in a family. Brief literature review ensues with the cases.

Magnesium 결핍이 항산화효소의 활성유도에 미치는 영향 (Effects of Magnesium Deficiency on Induction of Activity of Antioxidative Enzymes)

  • 김병철;이증주
    • 한국잡초학회지
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    • 제17권4호
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    • pp.431-438
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    • 1997
  • 유해(有害)한 활성산소종(活性酸素種)들을 생성하는 환경 stress에 대한 식물의 적응과정(適應過程)을 파악하기 위한 일환으로서, 호박을 식물재료로 공시하고 Mg이 결핍(缺乏)된 영양액(營養液)에서 재배하여 산화적(酸化的) stress 조건을 부여하였을 때, 나타나는 과산화(過酸化)의 피해정도와 항산화효소(抗酸化酵素)들의 활성변동(活性變動)을 조사하였다. 충분한 양의 Mg이 공급된 영양액에서 재배된 호박의 잎에서는 Mg, 엽록소(葉綠素), 단백질(蛋白質) 함량(含量) 및 항산화효소(抗酸化酵素)들의 활성(活性)이 다소 증가 되었거나 거의 일정하게 유지되었다. 그러나 Mg이 결핍(缺乏)된 영양액(營養液)에서 재배된 호박의 잎에서는 Mg, 엽록소(葉綠素), 단백질(蛋白質) 함량(含量)은 현저히 감소된 반면, 항산화효소(抗酸化酵素)인 AP, GR 및 SOD의 활성(活性)이 크게 증가되었다. 본 연구에서 얻어진 결과들은 식물에서 Mg의 결핍(缺乏)으로 이하여 해로운 활성산소종(活性酸素種)이 생성되며 동시에 이들 활성산소종(活性酸素種)의 유해(有害)한 작용을 소거(消去)하기 위한 항산화효소(抗酸化酵素)의 활성(活性)도 유도(誘導)되는 것을 시사한다.

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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
    • 한국생물물리학회:학술대회논문집
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    • 한국생물물리학회 2003년도 정기총회 및 학술발표회
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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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칼륨 결핍이 이탈리안 라이그라스 재생기간동안의 탄소와 절소의 대사에 미치는 영향 (Effects of Potassium Deficiency on C and N Metabolism during Regrowth of Italian Ryegrass (Lolium multiflorum L.))

  • 정우진;이복례;김대현;김길용;김태환
    • 한국초지조사료학회지
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    • 제21권2호
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    • pp.81-88
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    • 2001
  • To investigate C and N metabolisms in response to potassium-deficient stress during regrowth of Italian ryegrass(Lolium multiflorum L.), C and N metabolites were analyzed at day 0 (cutting date), 6, 12 and 24 days after defoliation. K-sufficient (control, +K) and K-absent (-K) nutrition solutions were applied from 7 days before defoliation, and continued for one cycle of 24 days-regrowth period. During 24 days of regrowth dry matter of regrowing shoots and remaining tissues were not significantly different between +K and -K treatment. In remaining stubble, all C compounds in both +K and -K treatment largely decreased (69% to 84% of the initial level) during the first 6 days of regrowth, and then rapidly recovered. The decline of soluble sugars and fructan in roots for the first 6 days much less in the -K medium. Amino acids, soluble and insoluble proteins in stubble also feel down during the first 6 days, thereafter actively replenished in both +K and -K treatment. The decline of nitrate in stubble prolonged to 12 days of regrowth. Initial amounts of all N compounds in roots were significantly lower in the -K medium. Higher accumulation of amino acids and soluble protein in roots in the -K medium was observed after 12 days of regrowth. In regrowing shoots, 3 all carbohydrates increased with a very similar pattern for both treatments. Nitrate was not significantly different between two treatments. Depress of soluble protein accumulation in -K medium was noteworthy after 12 days of regrowth. These results indicated that an active utilization of organic reserves occurred to support regrowth even under K deficient condition with a similar extent with K sufficient condition.

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지방산 산화 장애 제어를 통한 SREBP-1c 결핍의 소포체 스트레스 유발 비알콜성지방간 보호작용 (SREBP-1c Ablation Protects Against ER Stress-induced Hepatic Steatosis by Preventing Impaired Fatty Acid Oxidation)

  • 이영승;티모씨 에프 오스본;서영교;전태일
    • 생명과학회지
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    • 제31권9호
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    • pp.796-805
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    • 2021
  • 간 소포체(ER) 스트레스는 비알콜성지방간과 인슐린 저항성의 발달에 기여하고, unfolded protein response(UPR)의 구성요소는 지질 대사를 조절한다. 최근 연구에 따르면 ER 스트레스와 비정상적인 세포 지질 대사 사이의 연관성이 보고되었으며, 이 과정에서 지질 대사의 중심 조절자인 sterol regulatory element binding proteins(SREBPs)의 관련성이 확인되었다. 그러나 ER 스트레스 동안 지질 대사를 조절하는 SREBP의 정확한 역할과 비알콜성지방간에 대한 기여는 아직 밝혀지지 않았다. 본 연구에서 SREBP-1c 결핍은 UPR, 염증 및 지방산 산화 조절을 통해 ER 스트레스에 의해 유도된 비알콜성지방간으로부터 생쥐를 보호한다는 것을 보여준다. SREBP-1c는 inositol requiring kinase 1α (IRE1α) 발현을 직접적으로 조절하고 ER 스트레스에 의해 유도된 tumor necrosis factor-α의 활성화를 매개하여 peroxisome proliferator-activated receptor γ coactivator 1-α (PGC1α)의 감소와 그에 따른 지방산 산화의 장애를 유발한다. 그러나, SREBP-1c의 유전적 결핍은 이러한 현상을 보호하여 간 염증과 지방 축적을 완화시킨다. SREBP-1c 결핍이 ER 스트레스에 의해 유도된 염증 신호를 방지하는 메커니즘은 아직 밝혀지지 않았지만, SREBP-1c가 결핍된 Kupffer 세포에서 IRE1α 신호의 변화가 염증 신호에 관여할 수 있을 것으로 생각된다. 본 연구결과는 SREBP-1c가 ER 스트레스에 의해 유도된 비알콜성지방간에서 UPR 및 염증의 조절에 중요한 역할을 함을 시사한다.

한국농촌(韓國農材) 미취학아동(未就學兒童)의 철결핍성빈혈(鐵缺乏性貧血)에 관(關)한 연구(硏究) -충청남도(忠淸南道) 대덕군(大德郡) 유성면(儒城面) 상대리(上垈里)를 중심(中心)으로- (A Study on Iron Deficiency Anemia of Pre-School Children in Rural Area in Korea)

  • 채범석;이효은
    • Journal of Nutrition and Health
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    • 제3권3호
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    • pp.149-159
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    • 1970
  • This study is to investigate the status of anemia, especially iron deficiency anemia among pre-school children in rural area in Korea. The survey was conducted in Sang-dae Ri, Yusong Myon, Daedok Gun, Chung Chong Nam-Do from July 30 th to August 12th, 1968. The measurements were done of height, weight, hematologist and biochemical levels on ninety-two pre-school children, 47 male, and 45 female, one to six years of age. Hemoglobin was determined by the method of cyanmethemoglobin and hematocrit by micro hematocrit centrifuge. The determination of serum iron, iron-binding capacity was done by the method of Ramsay using bathophenanthroline and the serum albumin was determined by Biuret Reaction. The results of this study are as follows: 1) 54.4 percent of the pre-school children weighed less than 90 percent of the Korean General Standard Weight level. 2) The average hemoglobin level was $11.0{\pm}1.57gm/100ml$, 38.0 percent of the children were anemic with less than 1.0gm/100ml. Of the anemic children 60 percent were below the Korean General Standard Weight level. 3) 27.5 percent of the pre-school children were found to have below 32 percent of a hematocrit values and 28.0 percent showed less than 33 percent in M.C.H.C. These results showed that the incidence of hypochromic anemia in these pre-school children was high. 4) 37.9 percent of these children had a serum iron level less than $50{\mu}g/100ml\;and\;31.0\;percent\;had\;a\;TIBC\;above\;400{\mu}g$ while 48.3 percent showed a transferrin saturation lower than 15 percent. On the basis of these findings, it is concluded than the cause of this anemia was iron deficiency. 5) In this group there was a little evidence of low total serum protein levels. However, 10.4 percent of the children had a deficient serum albumin level, below 2.80 gm/100ml while 51.7 percent had a low level, less than 3.50gm/100ml, and 34.5 percent of the children had a low level of TIBC, less than $350{\mu}g/100ml$, and considering these facts, it is suggested that some of the anemias have a multiple causes through protein deficiency and repeated chronic infection apart from iron deficiency.

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Oncogenic RUNX3: A Link between p53 Deficiency and MYC Dysregulation

  • Date, Yuki;Ito, Kosei
    • Molecules and Cells
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    • 제43권2호
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    • pp.176-181
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    • 2020
  • The RUNX transcription factors serve as master regulators of development and are frequently dysregulated in human cancers. Among the three family members, RUNX3 is the least studied, and has long been considered to be a tumor-suppressor gene in human cancers. This idea is mainly based on the observation that RUNX3 is inactivated by genetic/epigenetic alterations or protein mislocalization during the initiation of tumorigenesis. Recently, this paradigm has been challenged, as several lines of evidence have shown that RUNX3 is upregulated over the course of tumor development. Resolving this paradox and understanding how a single gene can exhibit both oncogenic and tumor-suppressive properties is essential for successful drug targeting of RUNX. We propose a simple explanation for the duality of RUNX3: p53 status. In this model, p53 deficiency causes RUNX3 to become an oncogene, resulting in aberrant upregulation of MYC.

Secretagogin deficiency causes abnormal extracellular trap formation in microglia

  • Yu Gyung Kim;Do-Yeon Kim
    • International Journal of Oral Biology
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    • 제49권2호
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    • pp.34-41
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    • 2024
  • Extracellular traps (ETs), primarily composed of DNA and antibacterial peptides, are mainly secreted by neutrophils to inhibit pathogen spread and eliminate microorganisms. Recent reports suggest that microglia can also secrete ETs, and these microglial ETs are associated with various neurological conditions, including nerve injury, tumor microenvironment, and ischemic stroke. However, the components and functions of microglial ETs remain underexplored. Secretagogin (Scgn), a calcium-sensor protein, plays a crucial role in the release of peptide hormones, such as insulin, in endocrine cells; however, its function in immune cells, including microglia, is not well understood. Our study demonstrated that Scgn deficiency can lead to the formation of abnormal ETs. We hypothesized that this may involve the c-Jun N-terminal kinase-myeloperoxidase pathway and autophagy.