• Title/Summary/Keyword: Urea cycle

Search Result 94, Processing Time 0.025 seconds

Immunofluorescence Microscopy and Biochemical Characterization of Two Nuclear Envelope Proteins of Amoeba proteus by Using a Monoclonal Antibody (단항체를 이용한 아메바(Amoeba proteus) 의 2종 핵막 단백질에 대한 면역형광현미경적 및 생화학적 특성 조사)

  • 안태인;유시욱조양래
    • The Korean Journal of Zoology
    • /
    • v.34 no.1
    • /
    • pp.44-53
    • /
    • 1991
  • Distribution of the antigens during the cell cycle of amoebae was followed by immunof-luorescence microscopy using a monoclonal antibody against the nucleus as a probe. While the cells were in the interphase, the antigen was localized on the nucleus membrane. But it was dispersed all over the cytoplasm during mitosis and cytokinesis. The molecular weights of the immunoreacted antigens were 210 KD and 190 KD as determined by SDS PAGE and western blotting of the purified nuclei. The antigens were not soluble in non-ionic detergent, but were released from the nucleus by incubation with 0.05 M sodium carbonate, pH 10.6 or with 8 M urea at serial chemical extraction. Thus the antigens appeared to be peripheral proteins of the nurBeus envelope. The isoelectic point of both antigens was 7.64 as determined by 2 D PAGE and transfer blotting. Considering the peiipherd association with the nucleus membrane and the dispersed distribution during mitosis, the antigens could be lamin like proteins. Hourever, it appears also possible that they are the component molecules of the unusually structured aurous lamina of amoeba nucleus since they have the large molecular weight and the basic pl.

  • PDF

Effect of Low Adapted Temperature and Medium Composition on Growth and Erythropoietin (EPO) Production by Chinese Hamster Ovary Cells

  • Kim Na Young;Kim Jung Hoe;Kim Hong Jin
    • Archives of Pharmacal Research
    • /
    • v.28 no.2
    • /
    • pp.220-226
    • /
    • 2005
  • Temperature and medium composition were changed with the aim of increasing growth and erythropoietin (EPO) production in EPO-producing Chinese hamster ovary (CHO) cells. We used the CHO cell line, IBE, and its derivative, CO5, which over-expresses the first two enzymes of the urea cycle, carbamoyl phosphate synthetase I (CPS I) and ornithine transcar-bamoylase (OTC). When supplements were added to the medium at $33\;^{\circ}C$, the growth of IBE and CO5 cells increased by $27\%\;and;26\%$, respectively and the maximum yield of EPO was increased by $40\%$ in both cell lines. The absolute EPO concentration in the CO5 cells was always $55{\sim}60\%$ higher than in the IBE cells. In addition, when the two cell lines were continuously cultured with supplements at $33\;^{\circ}C$ until their growth rates approached those at $37\;^{\circ}C$, the growth rates of both IBE and CO5 cells increased by $54\%$ and their maximum EPO levels increased by up to $73\%\;and\;56\%$, respectively. Therefore, the growth and EPO expression levels of CO5 cells increased 2.2-fold and 2.6-fold, respectively, compared to those of the IBE cells. These results indicate that adaptation to lower temperature as well as medium supplementation could be important for improving cell growth and EPO production.

Neurological Outcome of Patients with Late-onset Ornithine Transcarbamylase Deficiency (지발형 오르니틴 트랜스카바미라제 결핍증 환자들의 신경학적 예후)

  • Jang, Kyung Mi;Hwang, Su-Kyeong
    • Journal of The Korean Society of Inherited Metabolic disease
    • /
    • v.22 no.1
    • /
    • pp.15-20
    • /
    • 2022
  • The most common urea cycle disorder is ornithine transcarbamylase deficiency. More than 80 percent of patients with symptomatic ornithine transcarbamylase deficiency are late-onset, which can present various phenotypes from infancy to adulthood. With no regards to the severity of the disease, characteristic fluctuating courses due to hyperammonemia may develop unexpectedly, and can be precipitated by various metabolic stressors. Late-onset ornithine transcarbamylase deficiency is not merely related to a type of genetic variation, but also to the complex relationship between genetic and environmental factors that result in hyperammonemia; therefore, it is difficult to predict the prevalence of neurological symptoms in late-onset ornithine transcarbamylase deficiency. Most common acute neurological manifestations include psychological changes, seizures, cerebral edema, and death; subacute neurological manifestations include developmental delays, learning disabilities, intellectual disabilities, attention-deficit/hyperactivity disorder, executive function deficits, and emotional and behavioral problems. This review aims to increase awareness of late-onset ornithine transcarbamylase deficiency, allowing for an efficient use of biochemical and genetic tests available for diagnosis, ultimately leading to earlier treatment of patients.

A Case of Carbamoyl Phosphate Synthetase 1 Deficiency with Novel Mutations in CPS1 Treated by Liver Cell Transplantation (신생아기에 새로운 CPS1 유전자 돌연변이가 확인되고, 간세포이식 시행 받은 Carbamoyl phosphatase synthetase 1 deficiency 1례)

  • Lee, Jisun;Yang, Aram;Kim, Jinsup;Park, Hyung-Doo;Lee, Sanghoon;Lee, Suk-Koo;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
    • /
    • v.17 no.1
    • /
    • pp.31-37
    • /
    • 2017
  • Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

  • PDF

Nutrient requirements in Hanwoo cows with artificial insemination: effects on blood metabolites and embryo recovery rate

  • Kang, Sung-Sik;Kim, Ui-Hyung;Han, Man-Hye;Cho, Sang-Rae
    • Journal of Animal Science and Technology
    • /
    • v.62 no.4
    • /
    • pp.449-459
    • /
    • 2020
  • Here, we investigated the effects of different nutrient requirements (NR) on blood metabolites, transferable embryo number after multiple superovulations with artificial insemination (AI), body condition score (BCS), and estrus cycle in Hanwoo cow. Nineteen Hanwoo cows were randomly divided into three groups (80%, 100%, and 120% NR, containing 6, 8, and 5 individuals, respectively) and fed based on the NR. In experiment 1, glucose, total cholesterol, triglyceride, blood urea nitrogen (BUN), aspartate aminotransferase (AST), alanine aminotransferase (ALT), non-esterified fatty acids (NEFA), albumin (ALB), and total protein (TP) were analyzed. In experiment 2, total number of recovered embryos and transferable embryos was examined after embryo recovery and multiple superovulations with AI. In experiment 3, body weight, BCS, and estrus cycle were examined. In experiment 1, total cholesterol was significantly different among the 80%, 100%, and 120% NR groups (126.5 ± 3.3, 152.6 ± 2.4, and 177.4 ± 1.8 mg/dL, respectively, p < 0.05). The triglyceride and BUN levels in the 120% NR group were significantly higher than those in the 80% and 100% groups (p < 0.05). The NEFA levels were significantly different among the 80%, 100%, and 120% NR groups (440.5 ± 18.2, 318.5 ± 23.1, and 195.1 ± 8.5 ЧEq/L, respectively, p < 0.05). The AST and TP levels in the 80% NR group were significantly lower than those in the 100% and 120% NR groups (p < 0.05). In experiment 2, the 120% NR group showed a higher percentage of transferable embryos than the 80% and 100% groups (p < 0.01). The mean body weight and BCS among the 80%, 100%, and 120% NR groups were significantly different (p < 0.05). The estrus cycle in the 80% NR group was delayed compared with the 100% and 120% NR groups (20.8 ± 0.2 and 21.2 ± 0.5 days, respectively). In conclusion, the blood metabolic tests proved that Hanwoo cows with 120% NR can produce a large number of transferable embryos. Thus, 120% NR is the appropriate feeding level for this type of cows as it results in the production of a large number of transferable embryos by multiple superovulations with AI.

Postpartum Reproductive Management Based on the Routine Farm Records of a Dairy Herd: Relationship between the Metabolic Parameters and Postpartum Ovarian Activity

  • Takagi, Mitsuhiro;Hirai, Toshiya;Moriyama, Naoki;Ohtani, Masayuki;Miyamoto, Akio;Wijayagunawardane, Missaka P.B.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • v.18 no.6
    • /
    • pp.787-794
    • /
    • 2005
  • The aim of this study was 1) to confirm the practical efficiency of a routine milk P4 monitoring system for postpartum reproductive management of a dairy herd, and 2) to evaluate the relationship between the blood metabolic profiles, milk quality and body weight of individual cows in the farm records, which may reflect the postpartum nutritional condition, and the time of postpartum resumption of ovarian activity of dairy cows. A total of 116 Holstein cows was used in the present study. First, during the period of Experiment 1, postpartum reproductive management based on weekly measured milk P4 concentration from individual cows was conducted. Compared with the reproductive records of the past two years without P4 monitoring, although the day from calving to first AI did not change, both the number of AI until pregnant (with P4; 1.9 times vs. without P4; 2.9 times) and the days open (with P4; 95.1 days vs. without P4; 135.8 days and 133.8 days) were significantly decreased. In Experiment 2, the measurement of blood constituents such as albumin, blood urea nitrogen, packed cell volume, ammonia, glucose, total cholesterol, non-esterified, AST and $\gamma$-GTP was performed on the blood samples taken once approximately 14 days postpartum, to monitor both health and nutritional conditions. The milk constituent parameters, such as milk protein (MP), milk fat (MF), SNF and lactose, collected from the monthly progeny test of individual cows, were used to monitor the postpartum nutritional status. Furthermore, the data obtained from the routine measurements of body weight were used to calculate the rate of peripartum body weight loss. The resumption day of the postpartum estrous cycle was assumed from the milk P4 profiles of individual cows. There was no clear relationship between each parameter from blood examination and those from resumption time. However, the cows had low values of MP, and SNF, which significantly affected the resumption of the postpartum estrous cycle. Similarly, a higher rate of body weight loss indicated a significant delay (more than 1 month) in the resumption of the postpartum estrous cycle, compared with the groups that had a medium or lower rate of body weight loss. The results of the present study demonstrated that the implementation of routine milk P4 monitoring-based postpartum reproductive management, together with milk quality parameters and routine BW data available in field conditions may be utilized as a practical approach for increasing the postpartum reproductive efficiency of a high yielding dairy herd.

Relationship between Transferable Embryos and Blood Urea Nitrogen, Glucose and Total Cholesterol Concentration in Hanwoo Donors (한우 공란우 이식기능 수정란수와 혈액내 요소태질소, 포도당 및 총콜레스테롤 수준과의 관계)

  • Son, Jun-Kyu;Choe, Chang-Yong;Cho, Sang-Rae;Lim, Hyun-Joo;Park, Seong-Jai;Baek, Kwang-Soo;Kim, Sang-Bum;Kwon, Eung-Gi;Jung, Yeon-Sub;Bok, Nan-Hee;Son, Dong-Soo
    • Reproductive and Developmental Biology
    • /
    • v.35 no.3
    • /
    • pp.335-339
    • /
    • 2011
  • The objective of this study was to investigate the relationship between concentration of urea nitrogen, glucose, cholesterol and number of transferable embryos for the purpose of improving reproductive performance in blood of Hanwoo donors. Fifty five, at random stages of the estrous cycle, received a CIDR. Four days later the animals were superovulated with a total of 28AU FSH (Antorin, 2AU=1 ml) administered twice daily in constant doses over 4 days. On the 3th administration of FSH, CIDR was withdrawn and 25 mg $PGF_2$ ${\alpha}$ was administered. Cows were artificially inseminated twice after estrous detection at 12 hr intervals. The cows received 100 ${\mu}g$ GnRH at the time of 1nd insemination. Embryos were recovered 7 or 8 days after the 1st insemination. Cows with BUN < 10, 11~18 and ${\geq}$19 mg/dl had number of transferable embryos of $4.32{\pm}1.3$, $5.8{\pm}1.8$ and $4.7{\pm}2.1$ respectively. The mean numbers of total ova from < 10 and 10${\leq}$ of corpora lutea(CL) was 8.9 and 14.3, respectively. The number of transferable embryos differed between < 10${\leq}$ CL was 4.8 and 5.6, respectively.

A Case of Ornithine Transcarbamylase Deficiency in 11-month-old Female who Presented Periodic Vomiting and Intermittent Consciousness Change (반복적인 구토 및 간헐적 의식 변화를 주소로 진단된 Ornithine Transcarbamylase Deficiency 여아 1례)

  • Kim, Jin Ah;Kim, Jin Sup;Huh, Rimm;Cho, Sung Yoon;Jin, Dong Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
    • /
    • v.15 no.3
    • /
    • pp.165-170
    • /
    • 2015
  • Ornithine transcarbamylase (OTC) deficiency is a rare X-linked genetic disorder of urea synthesis in newborns. It is the most common urea cycle disorder and leads to elevated levels of ammonia in the blood. Excessive ammonia can cause various symptoms, including vomiting, lethargy, and coma. Boys have a more serious form of OTC deficiency than girls. If not treated immediately, severe OTC deficiency can lead to neurologic abnormalities, hyperammonemic coma, and death. Because late-onset OTC deficiency, which is more common in girls, presents mild symptoms, it is easy to miss diagnosis and prompt treatment. We describe an 11-month-old girl who presented periodic vomiting, intermittent lethargy, and seizure. She was diagnosed with OTC deficiency by elevated serum ammonia and urine orotic acid levels. Genetic analysis of the OTC gene revealed a missense mutation in exon 5 (c.418G>C). We reported an experience of exact diagnosis and successful treatment of late-onset OTC deficiency in our patient.

The Distribution and Geomorphic Development of Alluvial Fans along the Bulguksa Fault System in Gyeongju and Ulsan City, Southeastern Korea (한국 남동부 경주 및 울산시 불국사단층선 지역의 선상지 분포와 지형발달)

  • 황상일;윤순옥
    • Journal of the Korean Geographical Society
    • /
    • v.36 no.3
    • /
    • pp.217-232
    • /
    • 2001
  • One of the molt debatable Issues on geomorphological study in Korea should be the discussion over the formation process of gent1e slope surfaces on the piedmont area. In this study, the characteristics of spatial distribution and the formation process of geomorphic surfaces were investigated by classifying the alluvial fans as three geomorphic surfaces alluvial the Bulguksa fault-line The fan surfaces, distributed along the west slue of Bulguksa Mts, consists the confluent alluvial fans continuously along the N-S direction The surfaces of Sincheon-Hyomun district juxtaposed to the Ulsan Bay must be infulenced by sea-level chance during the Quaternary Taken together, these observation suggests that the major four factors contributed to the fan formation 1) rather longer freeze-and-thaw cycle during the Glacial period. 2) the steep mountain slope along the west side of Bulguksa Mts.. which had been resulted from the horizont stress of EAst Sea 3)the tectolinear fault system developed by structural movement along the Bulguksa Fault-line valley. and 4) the erosion-labile characteristics of bedrock In this urea which is consisted of the Bulguksa granite and the sedimentary rock formed in Cretaceous period.

  • PDF

Gene Expression Profiling of Liver and Mammary Tissues of Lactating Dairy Cows

  • Baik, M.;Etchebarne, B.E.;Bong, J.;VandeHaar, M.J.
    • Asian-Australasian Journal of Animal Sciences
    • /
    • v.22 no.6
    • /
    • pp.871-884
    • /
    • 2009
  • Gene expression profiling is a useful tool for identifying critical genes and pathways in metabolism. The objective of this study was to determine the major differences in the expression of genes associated with metabolism and metabolic regulation in liver and mammary tissues of lactating cows. We used the Michigan State University bovine metabolism (BMET) microarray; previously, we have designed a bovine metabolism-focused microarray containing known genes of metabolic interest using publicly available genomic internet database resources. This is a high-density array of 70mer oligonucleotides representing 2,349 bovine genes. The expression of 922 genes was different at p<0.05, and 398 genes (17%) were differentially expressed by two-fold or more with 222 higher in liver and 176 higher in mammary tissue. Gene ontology categories with a high percentage of genes more highly expressed in liver than mammary tissues included carbohydrate metabolism (glycolysis, glucoenogenesis, propanoate metabolism, butanoate metabolism, electron carrier and donor activity), lipid metabolism (fatty acid oxidation, chylomicron/lipid transport, bile acid metabolism, cholesterol metabolism, steroid metabolism, ketone body formation), and amino acid/nitrogen metabolism (amino acid biosynthetic process, amino acid catabolic process, urea cycle, and glutathione metabolic process). Categories with more genes highly expressed in mammary than liver tissue included amino acid and sugar transporters and MAPK, Wnt, and JAK-STAT signaling pathways. Real-time PCR analysis showed consistent results with those of microarray analysis for all 12 genes tested. In conclusion, microarray analyses clearly identified differential gene expression profiles between hepatic and mammary tissues that are consistent with the differences in metabolism of these two tissues. This study enables understanding of the molecular basis of metabolic adaptation of the liver and mammary gland during lactation in bovine species.