![Molecular basis of hepatic carnitine palmitoyltransferase I deficiency](http://s1.studyres.com/store/data/015050650_1-854127437b85ea740c20db0bbd3f810b-300x300.png)
Molecular basis of hepatic carnitine palmitoyltransferase I deficiency
... zyme (CPT I) is localized in the outer-mitochondrial membrane and converts the fatty acyl-CoA ester to its carnitine ester. This carnitine ester is subsequently transported across the mitochondrial inner membrane by CAC which belongs to the mitochondrial carrier family. Once inside the mitochondrial ...
... zyme (CPT I) is localized in the outer-mitochondrial membrane and converts the fatty acyl-CoA ester to its carnitine ester. This carnitine ester is subsequently transported across the mitochondrial inner membrane by CAC which belongs to the mitochondrial carrier family. Once inside the mitochondrial ...
Identification of catalytically essential amino acid residues and immobilization Rumex
... to mitochondria has been reported for several plants [1 4 ], however it was suggested that the cytosolic activity was due to broken mitochondria. The enzyme is multiisoenzme in many plant tissues. T hus, GDH has been purified and characterized in microorganisms and higher plants. However, little inf ...
... to mitochondria has been reported for several plants [1 4 ], however it was suggested that the cytosolic activity was due to broken mitochondria. The enzyme is multiisoenzme in many plant tissues. T hus, GDH has been purified and characterized in microorganisms and higher plants. However, little inf ...
attached paper highlights
... mitochondrial number and size, concomitant with upregulation of genes associated with mitochondrial biogenesis.20 In the adult, this leads to a more modest increase in mitochondrial number, but with perturbation of mitochondrial ultrastructure and development of cardiomyopathy.20 Given the significan ...
... mitochondrial number and size, concomitant with upregulation of genes associated with mitochondrial biogenesis.20 In the adult, this leads to a more modest increase in mitochondrial number, but with perturbation of mitochondrial ultrastructure and development of cardiomyopathy.20 Given the significan ...
Open the publication - UEF Electronic Publications
... diabetes, certain cancers and mental problems, such as depression and schizophrenia16,19. Pgc1α, key regulator of cellular metabolism, has been identified as the key link between changes in metabolism and circadian clock. At the molecular level, the circadian clock represents a complex gene regulato ...
... diabetes, certain cancers and mental problems, such as depression and schizophrenia16,19. Pgc1α, key regulator of cellular metabolism, has been identified as the key link between changes in metabolism and circadian clock. At the molecular level, the circadian clock represents a complex gene regulato ...
13C MRS: An outstanding tool for metabolic studies
... C in specific positions have made it possible to follow in vivo and in vitro the activity of a large variety of metabolic pathways in cells, animals, and humans. These include glycolysis and the pentose phosphate pathway, glycogen synthesis and degradation, gluconeogenesis, the tricarboxylic acid cyc ...
... C in specific positions have made it possible to follow in vivo and in vitro the activity of a large variety of metabolic pathways in cells, animals, and humans. These include glycolysis and the pentose phosphate pathway, glycogen synthesis and degradation, gluconeogenesis, the tricarboxylic acid cyc ...
High pKa variability of cysteine residues in structural databases and
... Cysteine (Cys) is a very versatile amino acid, often found at the functional sites of proteins, where it serves a variety of structural and functional roles [1-3], including structural stabilization (disulfide bridges, metal coordination), catalysis (e.g. active site nucleophile in thiol-based oxido ...
... Cysteine (Cys) is a very versatile amino acid, often found at the functional sites of proteins, where it serves a variety of structural and functional roles [1-3], including structural stabilization (disulfide bridges, metal coordination), catalysis (e.g. active site nucleophile in thiol-based oxido ...
SODIUM-COUPLED TRANSPORTERS FOR KREBS CYCLE
... The metabolic intermediates of the Krebs or citric acid cycle constitute a group of organic anions that include dicarboxylates such as succinate and α-ketoglutarate and tricarboxylates such as citrate. The Krebs cycle intermediates are transported across plasma membranes of cells by secondary active ...
... The metabolic intermediates of the Krebs or citric acid cycle constitute a group of organic anions that include dicarboxylates such as succinate and α-ketoglutarate and tricarboxylates such as citrate. The Krebs cycle intermediates are transported across plasma membranes of cells by secondary active ...
as a PDF
... demands of biosynthetic processes change in a continuous manner with specific growth rate while the energy demand of maintenance processes remains constant (116). Thus, maintenance energy becomes an increasingly greater fraction of the total energy flow in the cell at low growth rates, and growth ef ...
... demands of biosynthetic processes change in a continuous manner with specific growth rate while the energy demand of maintenance processes remains constant (116). Thus, maintenance energy becomes an increasingly greater fraction of the total energy flow in the cell at low growth rates, and growth ef ...
Suberin biosynthesis in O. sativa: characterisation of a cytochrome
... An organism is defined to be alive when it features certain characteristics: It has to have the ability to reproduce itself, a metabolism to absorb and convert energy from the environment and to build its own components, it has to be discrete from its surrounding media, and maintain its homoeostasis ...
... An organism is defined to be alive when it features certain characteristics: It has to have the ability to reproduce itself, a metabolism to absorb and convert energy from the environment and to build its own components, it has to be discrete from its surrounding media, and maintain its homoeostasis ...
Reprint
... II (MPO-Fe4+-OH) and can oxidize AH by one-electron transfer with formation of A•-radicals (reaction 3, Fig. 1) [12, 16, 19]. Compound II is catalytically inactive in hypohalous acid formation, but, like Compound I, it can cause one-electron oxidation of substrate (AH) with regeneration of the nativ ...
... II (MPO-Fe4+-OH) and can oxidize AH by one-electron transfer with formation of A•-radicals (reaction 3, Fig. 1) [12, 16, 19]. Compound II is catalytically inactive in hypohalous acid formation, but, like Compound I, it can cause one-electron oxidation of substrate (AH) with regeneration of the nativ ...
Full-Text PDF
... Like peroxisomes, mitochondria are also dynamic organelles that continuously adapt their number, morphology, and function to prevailing environmental conditions [12]. In mammals, these organelles play a central role in many metabolic processes including–among others–adenosine triphosphate (ATP) gene ...
... Like peroxisomes, mitochondria are also dynamic organelles that continuously adapt their number, morphology, and function to prevailing environmental conditions [12]. In mammals, these organelles play a central role in many metabolic processes including–among others–adenosine triphosphate (ATP) gene ...
The effect of short chain fatty acids on glucose homeostasis
... the K+ channels and opens the Ca2+ channels in the membrane. The consequential influx of Ca2+ activates pyruvate dehydrogenase, leading to a higher conversion of pyruvate into acetyl coenzyme A (acetyl CoA), which raises the ATP/ADP ratio further through the citric acid cycle. The concentration of p ...
... the K+ channels and opens the Ca2+ channels in the membrane. The consequential influx of Ca2+ activates pyruvate dehydrogenase, leading to a higher conversion of pyruvate into acetyl coenzyme A (acetyl CoA), which raises the ATP/ADP ratio further through the citric acid cycle. The concentration of p ...
Analyzing Amino-Acid Sequences
... may have similar sequences of amino acids in their proteins and be closely related through evolution. Many researchers believe that the greater the similarity in the amino-acid sequences of two organisms, the more closely related they are in an evolutionary sense. Conversely, the greater the time th ...
... may have similar sequences of amino acids in their proteins and be closely related through evolution. Many researchers believe that the greater the similarity in the amino-acid sequences of two organisms, the more closely related they are in an evolutionary sense. Conversely, the greater the time th ...
The Effects of a Dual Challenge of Porcine Reproductive and
... I would like to express sincere gratitude to Dr. Robert Rhoads for allowing me the opportunity to be a part of his lab and to learn from his wealth of knowledge. I would like to thank him for his patience and guidance along my journey to pursue this degree. I would like to express my deepest appreci ...
... I would like to express sincere gratitude to Dr. Robert Rhoads for allowing me the opportunity to be a part of his lab and to learn from his wealth of knowledge. I would like to thank him for his patience and guidance along my journey to pursue this degree. I would like to express my deepest appreci ...
Nutrient transport into germinating Trichoderma
... apparent from plotting conidia diameter versus time that the diameter changes discontinuously, with a break of the curve at 8 h, the time when the first germ tubes appeared. After this, the biomass production accelerated (Fig. 1b). To identify factors that determine conidial swelling, the swelling o ...
... apparent from plotting conidia diameter versus time that the diameter changes discontinuously, with a break of the curve at 8 h, the time when the first germ tubes appeared. After this, the biomass production accelerated (Fig. 1b). To identify factors that determine conidial swelling, the swelling o ...
Functional Characterization of Nine Norway
... inclusion of these TPS into the TPS-d subfamily revealed that functional specialization of conifer TPS occurred before speciation of Pinaceae. Furthermore, based on TPS enclaves created by distinct branching patterns, the TPS-d subfamily is divided into three groups according to sequence similaritie ...
... inclusion of these TPS into the TPS-d subfamily revealed that functional specialization of conifer TPS occurred before speciation of Pinaceae. Furthermore, based on TPS enclaves created by distinct branching patterns, the TPS-d subfamily is divided into three groups according to sequence similaritie ...
THE INFLUENCE OF NUTRITIONAL PHOSPHATE DEPRIVATION ARABIDOPSIS THALIANA
... predicted to function in Pi scavenging, cell wall and ROS metabolism, proteolysis, and pathogen responses. The relationship between mRNA levels and relative amounts of selected secretome proteins was assessed. The results indicate that transcriptional control is but one of many factors contributing ...
... predicted to function in Pi scavenging, cell wall and ROS metabolism, proteolysis, and pathogen responses. The relationship between mRNA levels and relative amounts of selected secretome proteins was assessed. The results indicate that transcriptional control is but one of many factors contributing ...
A E M , Feb. 2004, p. 999–1007
... synthase (26) in E. coli grown in the presence of gluconate to accumulate MCL PHA. Coexpression of the E. coli tesA gene and the Pseudomonas oleovorans phaC1 gene led to the accumulation of MCL PHA copolymer composed of C6, C8, and C10 monomers (15). Coexpression of the U. californica thioesterase g ...
... synthase (26) in E. coli grown in the presence of gluconate to accumulate MCL PHA. Coexpression of the E. coli tesA gene and the Pseudomonas oleovorans phaC1 gene led to the accumulation of MCL PHA copolymer composed of C6, C8, and C10 monomers (15). Coexpression of the U. californica thioesterase g ...
Cloning and Polymorphisms of Yak Lactate Dehydrogenase b Gene
... of genetic polymorphisms of H subunit of LDH might give rise to 5 to 2 isozyme forms for LDH1 to LDH4, respectively [6]. In this study, we observed clearly the existence of multiple bands of LDH1 to LDH3 isozymes (Figure 1, lane 7). cDNA cloning of this sample demonstrated that its H subunit is enco ...
... of genetic polymorphisms of H subunit of LDH might give rise to 5 to 2 isozyme forms for LDH1 to LDH4, respectively [6]. In this study, we observed clearly the existence of multiple bands of LDH1 to LDH3 isozymes (Figure 1, lane 7). cDNA cloning of this sample demonstrated that its H subunit is enco ...
Carnitine: A Review - Society of Education~Agra
... r e q u i r e s i t will produce deficiency signs and symptoms. Many of the vitamins function as coenzymes (metabolic catalysts); others have no such role, but perform certain essential functions. Some vitamins deviate from the preceding definition in that they do not always need to be constituents ...
... r e q u i r e s i t will produce deficiency signs and symptoms. Many of the vitamins function as coenzymes (metabolic catalysts); others have no such role, but perform certain essential functions. Some vitamins deviate from the preceding definition in that they do not always need to be constituents ...
Prevention of Mitochondrial Oxidative Damage as a
... Metabolism strips electrons from fatty acids, sugars, and amino acids and accumulates them on the soluble electron carrier NADH and on protein-bound FADH2 (Fig. 1). The electrons are then passed down the mitochondrial respiratory chain to drive ATP synthesis by oxidative phosphorylation. As the elec ...
... Metabolism strips electrons from fatty acids, sugars, and amino acids and accumulates them on the soluble electron carrier NADH and on protein-bound FADH2 (Fig. 1). The electrons are then passed down the mitochondrial respiratory chain to drive ATP synthesis by oxidative phosphorylation. As the elec ...
IMD Program List of Disorders, Covered Drugs
... 1 new disorder has been added: Glucose transporter 1 (GLUT1) deficiency syndrome (GLUT1DS). Drugs and Supplements Approved disorder(s) have been added to 4 drugs/ supplements. DIN/PIN/etc. has been clarified for 5-hydroxytryptophan. Going forward, only 5hydroxytryptophan products with valid Na ...
... 1 new disorder has been added: Glucose transporter 1 (GLUT1) deficiency syndrome (GLUT1DS). Drugs and Supplements Approved disorder(s) have been added to 4 drugs/ supplements. DIN/PIN/etc. has been clarified for 5-hydroxytryptophan. Going forward, only 5hydroxytryptophan products with valid Na ...
Brønsted Acidity in Metal−Organic Frameworks
... containing several different kinds of metal ions within one SBU83 is highly desirable for developing stronger Brønsted acids based on bridging hydroxyl groups. Water molecules bound to metal sites could also result in Brønsted acidity, as exemplified by MIL-100.84,85 Mediumstrength Brønsted acidic sit ...
... containing several different kinds of metal ions within one SBU83 is highly desirable for developing stronger Brønsted acids based on bridging hydroxyl groups. Water molecules bound to metal sites could also result in Brønsted acidity, as exemplified by MIL-100.84,85 Mediumstrength Brønsted acidic sit ...
Metabolism
![](https://commons.wikimedia.org/wiki/Special:FilePath/ATP-3D-vdW.png?width=300)
Metabolism (from Greek: μεταβολή metabolē, ""change"") is the set of life-sustaining chemical transformations within the cells of living organisms. These enzyme-catalyzed reactions allow organisms to grow and reproduce, maintain their structures, and respond to their environments. The word metabolism can also refer to all chemical reactions that occur in living organisms, including digestion and the transport of substances into and between different cells, in which case the set of reactions within the cells is called intermediary metabolism or intermediate metabolism.Metabolism is usually divided into two categories: catabolism, the breaking down of organic matter by way of cellular respiration, and anabolism, the building up of components of cells such as proteins and nucleic acids. Usually, breaking down releases energy and building up consumes energy.The chemical reactions of metabolism are organized into metabolic pathways, in which one chemical is transformed through a series of steps into another chemical, by a sequence of enzymes. Enzymes are crucial to metabolism because they allow organisms to drive desirable reactions that require energy that will not occur by themselves, by coupling them to spontaneous reactions that release energy. Enzymes act as catalysts that allow the reactions to proceed more rapidly. Enzymes also allow the regulation of metabolic pathways in response to changes in the cell's environment or to signals from other cells.The metabolic system of a particular organism determines which substances it will find nutritious and which poisonous. For example, some prokaryotes use hydrogen sulfide as a nutrient, yet this gas is poisonous to animals. The speed of metabolism, the metabolic rate, influences how much food an organism will require, and also affects how it is able to obtain that food.A striking feature of metabolism is the similarity of the basic metabolic pathways and components between even vastly different species. For example, the set of carboxylic acids that are best known as the intermediates in the citric acid cycle are present in all known organisms, being found in species as diverse as the unicellular bacterium Escherichia coli and huge multicellular organisms like elephants. These striking similarities in metabolic pathways are likely due to their early appearance in evolutionary history, and their retention because of their efficacy.