• Title/Summary/Keyword: Metabolic Cycle

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Clinical Manifestations of Inborn Errors of the Urea Cycle and Related Metabolic Disorders during Childhood

  • Endo, Fumio;Matsuura, Toshinobu;Yanagita, Kaede;Matsuda, Ichiro
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.5 no.1
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    • pp.76-87
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    • 2005
  • Various disorders cause hyperammonemia during childhood. Amongthem are those caused by inherited defects in urea synthesis and related metabolic pathways. These disorders can be grouped into two types: disorders of the enzymes that comprise the urea cycle, and disorders of the transporters or metabolites of theamino acids related to the urea cycle. Principal clinical features of these disorders are caused by elevated levels of blood ammonium. Additional disease-specific symptoms are related to the particular metabolic defect. These specific clinical manifestations are often due to an excess or lack of specific amino acids. Treatment of urea cycle disorders and related metabolic diseases consists of nutritional restriction of proteins, administration of specific amino acids, and use of alternative pathways for discarding excess nitrogen. Although combinations of these treatments are extensively employed, the prognosis of severe cases remains unsatisfactory. Liver transplantation is one alternative for which a better prognosis is reported.

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Applications of Metabolic Modeling to Drive Bioprocess Development for the Production of Value-added Chemicals

  • Mahadevan, Radhakrishnan;Burgard, Anthony P.;Famili, Iman;Dien, Steve Van;Schilling, Christophe H.
    • Biotechnology and Bioprocess Engineering:BBE
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    • v.10 no.5
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    • pp.408-417
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    • 2005
  • Increasing numbers of value added chemicals are being produced using microbial fermentation strategies. Computational modeling and simulation of microbial metabolism is rapidly becoming an enabling technology that is driving a new paradigm to accelerate the bioprocess development cycle. In particular, constraint-based modeling and the development of genome-scale models of industrial microbes are finding increasing utility across many phases of the bioprocess development workflow. Herein, we review and discuss the requirements and trends in the industrial application of this technology as we build toward integrated computational/experimental platforms for bioprocess engineering. Specifically we cover the following topics: (1) genome-scale models as genetically and biochemically consistent representations of metabolic networks; (2) the ability of these models to predict, assess, and interpret metabolic physiology and flux states of metabolism; (3) the model-guided integrative analysis of high throughput 'omics' data; (4) the reconciliation and analysis of on- and off-line fermentation data as well as flux tracing data; (5) model-aided strain design strategies and the integration of calculated biotransformation routes; and (6) control and optimization of the fermentation processes. Collectively, constraint-based modeling strategies are impacting the iterative characterization of metabolic flux states throughout the bioprocess development cycle, while also driving metabolic engineering strategies and fermentation optimization.

Investigation of the Central Carbon Metabolism of Sorangium cellulosum: Metabolic Network Reconstruction and Quantification of Pathway Fluxes

  • Bolten, Christoph J.;Heinzle, Elmar;Muller, Rolf;Wittmann, Christoph
    • Journal of Microbiology and Biotechnology
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    • v.19 no.1
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    • pp.23-36
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    • 2009
  • In the present work, the metabolic network of primary metabolism of the slow-growing myxobacterium Sorangium cellulosum was reconstructed from the annotated genome sequence of the type strain So ce56. During growth on glucose as the carbon source and asparagine as the nitrogen source, So ce56 showed a very low growth rate of $0.23\;d^{-1}$, equivalent to a doubling time of 3 days. Based on a complete stoichiometric and isotopomer model of the central metabolism, $^{13}C$ metabolic flux analysis was carried out for growth with glucose as carbon and asparagine as nitrogen sources. Normalized to the uptake flux for glucose (100%), cells recruited glycolysis (51%) and the pentose phosphate pathway (48%) as major catabolic pathways. The Entner-Doudoroff pathway and glyoxylate shunt were not active. A high flux through the TCA cycle (118%) enabled a strong formation of ATP, but cells revealed a rather low yield for biomass. Inspection of fluxes linked to energy metabolism revealed that S. cellulosum utilized only 10% of the ATP formed for growth, whereas 90% is required for maintenance. This explains the apparent discrepancy between the relatively low biomass yield and the high flux through the energy-delivering TCA cycle. The total flux of NADPH supply (216%) was higher than the demand for anabolism (156%), indicating additional reactions for balancing of NADPH. The cells further exhibited a highly active metabolic cycle, interconverting $C_3$ and $C_4$ metabolites of glycolysis and the TCA cycle. The present work provides the first insight into fluxes of the primary metabolism of myxobacteria, especially for future investigation on the supply of cofactors, building blocks, and energy in myxobacteria, producing natural compounds of biotechnological interest.

Development of Cucumber Cotyledon in View of Metabolic Pathways and Organelle (세포내 소기관과 물질대사의 관점에서 오이 떡잎의 발달)

  • Kim, Dae-Jae
    • Journal of Life Science
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    • v.31 no.8
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    • pp.778-785
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    • 2021
  • The germination of cucumber seeds begins with the degradation of reserved oil to fatty acids within the lipid body, which are then further metabolized to acyl-CoA. The acyl-CoA moves from the lipid body to the glyoxysome following β-oxidation for the production of acetyl-CoA. As an initial carbon source supplier, acetyl-CoA is an essential molecule in the glyoxylate cycle within the glyoxysome, which produces the metabolic intermediates of citrate and malate, among others. The glyoxylate cycle is a necessary metabolic pathway for oil seed plant germination because it produces the metabolic intermediates for the tricarboxylic acid (TCA) cycle and for gluconeogenesis, such as the oxaloacetate, which moves to the cytosol for the initiation of gluconeogenesis by phophoenolpyruvate carboxykinase (PEPCK). Following reserved oil mobilization, the production and transport of various metabolic intermediates are involved in the coordinated operation and activation of multiple metabolic pathways to supply directly usable carbohydrate in the form of glucose. Furthermore, corresponding gene expression regulation compatibly transforms the microbody to glyoxysome, which contains the organelle-specific malate synthase (MS) and isocitrate lyase (ICL) enzymes during oil seed germination. Together with glyoxylate cycle, carnitine, which mediates the supplementary route of the acetyl-CoA transport mechanism via the mitochondrial BOU (A BOUT DE SOUFFLE) system, possibly plays a secondary role in lipid metabolism for enhanced plant development.

Metabolic Rebalancing of CR6 Interaction Factor 1-Deficient Mouse Embryonic Fibroblasts: A Mass Spectrometry-Based Metabolic Analysis

  • Tadi, Surendar;Kim, Soung Jung;Ryu, Min Jeong;Park, Taeseong;Jeong, Ji-Seon;Kim, Young Hwan;Kweon, Gi Ryang;Shong, Minho;Yim, Yong-Hyeon
    • Bulletin of the Korean Chemical Society
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    • v.34 no.1
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    • pp.35-41
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    • 2013
  • Metabolic analysis of CR6 interacting factor 1 (Crif1) deficient mouse embryonic fibroblasts with impaired oxidative phosphorylation has been carried out using LC-MS/MS and GC-MS methods. Metabolic profiles of the Crif1 deficient cells were comprehensively obtained for the first time. Loss of oxidative phosphorylation functions in mitochondria resulted in cancer-like metabolic reprogramming with consumption of majority of glucose carbon from up-regulated glycolysis to produce lactate, suppressed utilization of glucose carbon in the TCA cycle, increased amounts of amino acids. The changes in metabolic profile of the Crif1 deficient cells are most probably a consequence of metabolic reprogramming to meet the needs of energy balance and anabolic precursors in compensation for the loss of major oxidative phosphorylation functions.

Thermoregulation on Menstrual Cycle -Effects of Ambient Temperatures- (생리주기에 따른 체온조절에 관한 연구 -환경온도의 영향을 중심으로-)

  • 황수경;최정화
    • Journal of the Korean Society of Clothing and Textiles
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    • v.25 no.2
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    • pp.339-349
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    • 2001
  • This study was investigated the effects of ambient temperatures and menstrual cycle on Resting Metabolic Rate(RMR), Rectal Temperature(Tre), Skin(forehead, chest, abdomen, forearm, hand, thigh, leg, foot) Temperatures, and subjective thermal sensations in 8 young Korean females(ages 22-25, voluntarily). The Tre and the Skin Temperatures were measured in once every five minute for one hour. RMR was measured three times at 30 minutes intervals by indirect calorimetry. All measurements were gathered during Luteal Phase(LP), Menstruation(M), and Follicular Phase(FP) at two levels of ambient temperatures; low(17~21$^{\circ}C$) and middle(21.1~$25^{\circ}C$). LP were the highest values during FP and M in RMR, Tre, forehead temperature, chest temperature and abdomen temperature, while the leg(leg and foot) and arm(forearm and hand) temperatures were higher during FP rather than during LP at each ambient temperature. The downward curve of Tre in the experiment was larger during FP than LP. The values in subjective thermal sensations were most comfortable during LP than M and FP at each ambient temperature. The LP-FP differences in core and mean skin temperatures, and resting metabolic rate, were more significant at middle ambient temperatures than at low ambient temperatures.

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Biological Clock and Ultradian Metabolic Oscillation in Saccharomyces cerevisiae (Saccharomyces cerevisiae의 생물시계와 초단기 대사진동)

  • Kwon, Chong Suk;Sohn, Ho-Yong
    • Journal of Life Science
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    • v.28 no.8
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    • pp.985-991
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    • 2018
  • Biological clocks are the basis of temporal control of metabolism and behavior. These clocks are characterized by autonomous free-running oscillation and temperature compensation and are found in animals, plants, and microorganisms. To date, various biological clocks have been reported. These include clocks governing hibernation, sleep/wake, heartbeat, and courtship song. These clocks can be differentiated by the period of rhythms, for example, infradian rhythms (> 24-hr period), circadian rhythms (24-hr period), and ultradian rhythms (< 24-hr period). In yeast (Saccharomyces cerevisiae), at least five different autonomous oscillations have been reported; (1) glycolytic oscillations (T = 1~30 min), (2) cell cycle-dependent oscillations (T = 2~16 hr), (3) ultradian metabolic oscillations (T = 15~50 min), (4) yeast colony oscillations (T = a few hours), and (5) circadian oscillations (T = 24 hr). In this review, we discuss studies on oscillators, pacemakers, and synchronizers, in addition to the application of biological clocks, to demonstrate the nature of autonomous oscillations, especially ultradian metabolic oscillations of S. cerevisiae.

The Effect of Sleep Loss on Energy and Metabolism (호르몬수면상실이 에너지와 대사에 미치는 영향)

  • Kang, Seung-Gul
    • Sleep Medicine and Psychophysiology
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    • v.19 no.1
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    • pp.5-10
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    • 2012
  • The release of hormones and the metabolism of human body are controlled by the circadian rhythm related to sleep-wake cycle. Growth hormone, prolactin, thyroid stimulating hormone, cortisol, glucose, and insulin-secretion rates fluctuate according to the sleep-wake cycle. In addition, sleep is related to the appetite regulation and carbohydrate and other energy metabolism. Hypocretin (orexin), an excitatory neuropeptide, regulates waking and diet intake, and the poor sleep increases diet intake. The short sleep duration increases one's body mass index and impairs the function of the endocrine and metabolism, causing increases in the risk of glucose intolerance and diabetes. The poor sleep quality and sleep disorders have similar impact on the metabolic function. In short, the sleep loss and the poor quality of sleep have a detrimental effect on the endocrine and energy metabolism. The improvement of sleep quality by the future research and appropriate clinical treatment would contribute to the decrease of the metabolic diseases such as diabetes.