• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제10권1호
• /
• pp.60-65
• /
• 2007

저자들은 가족 내에서 아동학대로 인해 발생한 치명적인 중증 영양실조를 보인 5세 여아에 동반된 빈혈과 구리 결핍증을 WHO 치료 지침과 구리 보충요법으로 호전시킨 1예를 치험하였기에 보고하는 바이다.

• Molecules and Cells
• /
• 제20권2호
• /
• pp.228-234
• /
• 2005

Caenorhabditis elegans him-6 mutants, which show a high incidence of males and partial embryonic lethality, are defective in the orthologue of human Bloom's syndrome protein (BLM). When strain him-6(e1104) containing a missense him-6 mutation was irradiated with ${\gamma}$-rays during germ cell development or embryogenesis, embryonic lethality was higher than in the wild type, suggesting a critical function of the wild type gene in mitotic and pachytene stage germ cells as well as in early embryos. Even in the absence of ${\gamma}$-irradiation, apoptosis was elevated in the germ cells of the him-6 strain and this increase was dependent on a functional p53 homologue (CEP-1), suggesting that spontaneous DNA damage accumulates due to him-6 deficiency. However, induction of germline apoptosis by ionizing radiation was not significantly affected by the deficiency, indicating that HIM-6 has no role in the induction of apoptosis by exogenous DNA damage. We conclude that the C. elegans BLM orthologue is involved in DNA repair in promeiotic cells undergoing homologous recombination, as well as in actively dividing germline and somatic cells.

• 한국산업보건학회지
• /
• 제29권1호
• /
• pp.42-49
• /
• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제25권1호
• /
• pp.79-86
• /
• 2022

Purpose: To determine the effect of zinc deficiency on fecal protein, electrolyte, and short-chain fatty acid levels in both heat-stable (ST) and heat-labile (LT) enterotoxigenic Escherichia coli (ETEC)-induced diarrhea in rats. Methods: Albino rats, weighing 100 to 150 g, were divided into 2 groups, with 15 animals each: non-zinc and zinc-deficient. These two groups were sub-divided into three sub-groups with five rats each: control (saline); LT-ETEC; and ST-ETEC. Sodium phytate (30 mmol/L) was added to the animals' water to induce zinc deficiency, while diarrhea was induced using 5×10⁹ ETEC cells/mL. Fecal protein levels were estimated using the Bradford method, while sodium and potassium levels were determined using atomic absorption spectrophotometry. Short-chain fatty acids were measured using gas chromatography-mass spectrometry. Results: Among the non-zinc and zinc-deficient groups, there were significant increases (p=0.04), (p=0.03) in fecal protein concentrations (mg/mL) in the LT-ETEC- (4.50±0.33), (6.50±0.26) and ST-ETEC- (3.85±0.19), (5.98±0.32) induced groups compared to the control groups (2.60±0.52), (3.50±0.11) respectively. Fecal sodium and potassium levels (mg/L) were significantly (p=0.029) increased in non-zinc-deficient rats induced with LT-ETEC (9.35±0.95, 1.05±0.48), and ST-ETEC (9.96±1.02, 1.21±0.45) compared with the control group (8.07±0.44, 0.47±0.17) but the increase were not statistically significant (p=0.059) in the zinc deficient rat groups. Fecal acetate and propionate levels (mg/g) significantly (p=0.032) increased when induced with LT-ETEC and ST-ETEC in non-zinc and zinc-deficient groups compared with the control groups. Conclusion: Zinc deficiency among rats with ETEC-induced diarrhea elevated fecal protein loss but may not have an effect on fecal sodium, potassium and short-chain fatty acid levels.

• Clinical and Experimental Pediatrics
• /
• 제59권sup1호
• /
• pp.29-31
• /
• 2016

Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures, one of the main clinical features of GLUT-1 deficiency, usually develop during infancy. Most patients experience brief and subtle myoclonic jerk and focal seizures that evolve into a mixture of different types of seizures, such as generalized tonic-clonic, absence, myoclonic, and complex partial seizures. Here, we describe the case of a patient with GLUT-1 deficiency who developed infantile spasms and showed delayed development at 6 months of age. She had intractable epilepsy despite receiving aggressive antiepileptic drug therapy, and underwent a metabolic workup. Cerebrospinal fluid (CSF) examination showed CSF-glucose-to-blood-glucose ratio of 0.38, with a normal lactate level. Bidirectional sequencing of SLC2A1 identified a missense mutation (c.1198C>T) at codon 400 (p.Arg400Cys) of exon 9.

• Journal of Plant Biology
• /
• 제11권1호
• /
• pp.15-21
• /
• 1968

Chlorella ellipsoidea were grown in a Mg-free medium. Aliquots of the algal cell were taken out at the beginning and predetermined time intervals during the culture and were analyzed the contents of phosphate in various fractions of the cell constituents. The results obtained were compared with those of the control. When Chlorella cells were grown in a Mg-free medium, the contents of phosphate in the DNA protein, RNA-olyphosphate complex, nucleotidic-lbileP, and PCA-soluble, fractions decreased compared with those of the control, while the content of acid insoluble polyphosphate increased significantly. On the otherhand, RNA-P and lipid-P showed the tendency of decrease at the early stage of the culture, but they were increased more than those in the control as culture proceeds. It is showed that phosphate turnover from acid-insoluble polyphosphate into DNA, protein, and RNA-polyphosphate complex was inhibited by magnesium-deficiency of the cells.

• Journal of Microbiology and Biotechnology
• /
• 제18권8호
• /
• pp.1368-1376
• /
• 2008

In our previous study, the expression of active H-ferritins in Saccharomyces cerevisiae was found to reduce cell growth and reactive oxygen species (ROS) generation upon exposure to oxidative stress; such expression enhanced that of high-affinity iron transport genes (FET3 and FTR1). The results suggested that the recombinant cells expressing H-ferritins induced cytosolic iron depletion. The present study analyzes metabolic changes under these circumstances via proteomic methods. The YGH2 yeast strain expressing A-ferritin, the YGH2-KG (E62K and H65G) mutant strain, and the YGT control strain were used. Comparative proteomic analysis showed that the synthesis of 34 proteins was at least stimulated in YGH2, whereas the other 37 proteins were repressed. Among these, the 31 major protein spots were analyzed via nano-LC/MS/MS. The increased proteins included major heat-shock proteins and proteins related to endoplasmic reticulum-associated degradation (ERAD). On the other hand, the proteins involved with folate metabolism, purine and methionine biosynthesis, and translation were reduced. In addition, we analyzed the insoluble protein fractions and identified the fragments of Idh1p and Pgk1p, as well as several ribosomal assembly-related proteins. This suggests that intracellular iron depletion induces imperfect translation of proteins. Although the proteins identified above result from changes in iron metabolism (i.e., iron deficiency), definitive evidence for iron-related proteins remains insufficient. Nevertheless, this study is the first to present a molecular model for iron deficiency, and the results may provide valuable information on the regulatory network of iron metabolism.

• Journal of Animal Science and Technology
• /
• 제58권8호
• /
• pp.29.1-29.10
• /
• 2016

Background: It is known that large dogs who are fed lamb and rice diets are at increased risk to develop taurine-deficiency-induced dilated cardiomyopathy. Since dogs obligatorily conjugate bile acids (BA) with taurine, we determined whether rice bran (RB) or other fibers (cellulose; CL, beet pulp; BP) would affect BA excretion and/or the taurine status of dogs. Results: Eighteen medium/large mixed-breed dogs were given purified diets containing CL, BP, or RB for 12 weeks. Taurine concentrations in plasma and whole blood were significantly decreased at week 12. The BP group, compared to the CL or RB groups, showed significantly lower taurine concentrations in plasma ($6.5{\pm}0.5$ vs $20.4{\pm}3.9$ and $13.1{\pm}2.0{\mu}mol/L$, respectively, P < 0.01, $mean{\pm}SEM$) and in whole blood ($79{\pm}10$ vs $143{\pm}14$ and $127{\pm}14{\mu}mol/L$, respectively, P < 0.01), lower apparent protein digestibility ($81.9{\pm}0.6$ vs $88.8{\pm}0.6$ and $88.1{\pm}1.2%$, respectively, P < 0.01), and higher BA excretions ($5.6{\pm}0.1$ vs $3.4{\pm}0.5$ and $3.4{\pm}0.4{\mu}mol/g$ feces, respectively, P < 0.05) at week 12. Conclusions: These results do not support the hypothesis that RB is likely to be a primary cause of lamb meal and rice diets, increasing the risk of taurine deficiency in large dogs. However these indicate that BP may contribute to a decrease taurine status in dogs by increasing excretion of fecal BA and decreasing protein digestibility, thus decreasing the bioavailability of sulfur amino acids, the precursors of taurine.

• 한국잡초학회지
• /
• 제17권4호
• /
• pp.431-438
• /
• 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.

• Molecules and Cells
• /
• 제37권11호
• /
• pp.777-784
• /
• 2014

Epidemiologic studies have shown that low-density lipoprotein cholesterol (LDL-C) is a strong risk factor, whilst high-density lipoprotein cholesterol (HDL-C) reduces the risk of coronary heart disease (CHD). Therefore, strategies to manage dyslipidemia in an effort to prevent or treat CHD have primarily attempted at decreasing LDL-C and raising HDL-C levels. Cholesteryl ester transfer protein (CETP) mediates the exchange of cholesteryl ester for triglycerides between HDL and VLDL and LDL. We have published the first report indicating that a group of Japanese patients who were lacking CETP had extremely high HDL-C levels, low LDL-C levels and a low incidence of CHD. Animal studies, as well as clinical and epidemiologic evidences, have suggested that inhibition of CETP provides an effective strategy to raise HDL-C and reduce LDL-C levels. Four CETP inhibitors have substantially increased HDL-C levels in dyslipidemic patients. This review will discuss the current status and future prospects of CETP inhibitors in the treatment of CHD. At present anacetrapib by Merck and evacetrapib by Eli Lilly are under development. By 100mg of anacetrapib HDL-C increased by 138%, and LDL-C decreased by 40%. Evacetrapib 500 mg also showed dramatic 132% increase of HDL-C, while LDL-C decreased by 40%. If larger, long-term, randomized, clinical end point trials could corroborate other findings in reducing atherosclerosis, CETP inhibitors could have a significant impact in the management of dyslipidemic CHD patients. Inhibition of CETP synthesis by antisense oligonucleotide or small molecules will produce more similar conditions to human CETP deficiency and may be effective in reducing atherosclerosis and cardiovascular events. We are expecting the final data of prospective clinical trials by CETP inhibitors in 2015.