Reaction Process: PlantCyc:MGUTTATUS_PWY-5071

Tetrahydrobiopterin

C9H15N5O3 (241.11748400000002)

Tetrahydrobiopterin (CAS: 17528-72-2), also known as BH4, is an essential cofactor in the synthesis of neurotransmitters and nitric oxide (PMID: 16946131). In fact, it is used by all three human nitric-oxide synthases (NOS) eNOS, nNOS, and iNOS as well as the enzyme glyceryl-ether monooxygenase. It is also essential in the conversion of phenylalanine into tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine into L-dopa by the enzyme tyrosine hydroxylase; and the conversion of tryptophan into 5-hydroxytryptophan via tryptophan hydroxylase. Specifically, tetrahydrobiopterin is a cofactor for tryptophan 5-hydroxylase 1, tyrosine 3-monooxygenase, and phenylalanine hydroxylase, all of which are essential for the formation of the neurotransmitters dopamine, noradrenaline, and adrenaline. Tetrahydrobiopterin has been proposed to be involved in the promotion of neurotransmitter release in the brain and the regulation of human melanogenesis. A defect in BH4 production and/or a defect in the enzyme dihydropteridine reductase (DHPR) causes phenylketonuria type IV, as well as dopa-responsive dystonias. BH4 is also implicated in Parkinsons disease, Alzheimers disease, and depression. Tetrahydrobiopterin is present in probably every cell or tissue of higher animals. On the other hand, most bacteria, fungi and plants do not synthesize tetrahydrobiopterin (Wikipedia). A - Alimentary tract and metabolism > A16 - Other alimentary tract and metabolism products > A16A - Other alimentary tract and metabolism products > A16AX - Various alimentary tract and metabolism products C26170 - Protective Agent > C275 - Antioxidant Tetrahydrobiopterin ((Rac)-Sapropterin) is a cofactor of the aromatic amino acid hydroxylases enzymes and also acts as an essential cofactor for all nitric oxide synthase (NOS) isoforms.

Oxygen

O2 (31.98983)

Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earths crust. Diatomic oxygen gas constitutes 20.9\\% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70\\% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131) [HMDB]. Oxygen is found in many foods, some of which are soy bean, watermelon, sweet basil, and spinach. Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earths crust. Diatomic oxygen gas constitutes 20.9\\% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70\\% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131). V - Various > V03 - All other therapeutic products > V03A - All other therapeutic products > V03AN - Medical gases

4a-Hydroxytetrahydrobiopterin

C9H15N5O4 (257.11239900000004)

Tetrahydrobiopterin (BH4) is essential for catalyzing the conversion of phenylalanine into tyrosine by phenylalanine hydroxylase. During this physiological reaction, the oxidation of BH4 creates 4a-hydroxytetrahydropterin (CAS: 70110-58-6) intermediates and hydrogen peroxide is formed. The hydrogen peroxide and the hydroxytetrahydropterin can both be derived from alternate breakdown routes of a common precursor, the corresponding 4a-hydroperoxytetrahydropterin (PMID: 8323303). Tetrahydrobiopterin (BH4) is essential to catalyze the conversion of phenylalanine to tyrosine by phenylalanine hydroxylase. During this physiological reaction, the oxidation of BH4 creates 4a-hydroxytetrahydropterin intermediates and hydrogen peroxide is formed. The hydrogen peroxide and the hydroxytetrahydropterin can both derive from alternate routes of breakdown of a common precursor, the corresponding 4a-hydroperoxytetrahydropterin. (PMID 8323303) [HMDB]

Ammonium

H4N+ (18.0343724)

Ammonium, also known as ammonium(1+) or nh4+, is a member of the class of compounds known as homogeneous other non-metal compounds. Homogeneous other non-metal compounds are inorganic non-metallic compounds in which the largest atom belongs to the class of other nonmetals. Ammonium can be found in a number of food items such as irish moss, sago palm, sorghum, and malabar spinach, which makes ammonium a potential biomarker for the consumption of these food products. Ammonium can be found primarily in blood and sweat. Ammonium exists in all living species, ranging from bacteria to humans. In humans, ammonium is involved in the the oncogenic action of 2-hydroxyglutarate. Ammonium is also involved in a couple of metabolic disorders, which include the oncogenic action of d-2-hydroxyglutarate in hydroxygluaricaciduria and the oncogenic action of l-2-hydroxyglutarate in hydroxygluaricaciduria. Moreover, ammonium is found to be associated with n-acetylglutamate synthetase deficiency. The ammonium cation is a positively charged polyatomic ion with the chemical formula NH+ 4. It is formed by the protonation of ammonia (NH3). Ammonium is also a general name for positively charged or protonated substituted amines and quaternary ammonium cations (NR+ 4), where one or more hydrogen atoms are replaced by organic groups (indicated by R) . Ammonium is an important source of nitrogen for many plant species, especially those growing on hypoxic soils. However, it is also toxic to most crop species and is rarely applied as a sole nitrogen source. The ammonium (more obscurely: aminium) cation is a positively charged polyatomic cation with the chemical formula NH4+. It is formed by the protonation of ammonia (NH3). Ammonium is also a general name for positively charged or protonated substituted amines and quaternary ammonium cations (NR4+), where one or more hydrogen atoms are replaced by organic radical groups (indicated by R). Ammonium is found to be associated with N-acetylglutamate synthetase deficiency, which is an inborn error of metabolism.

cinnamate

C9H7O2- (147.0446022)

A member of the class of cinnamates that results from the deprotonation of the carboxy group of cinnamic acid.

(2S)-2-azaniumyl-3-phenylpropanoate

C9H11NO2 (165.0789746)

(2S)-2-ammonio-3-(4-hydroxyphenyl)propanoate

C9H11NO3 (181.0738896)

(2S)-2-azaniumyl-3-(3,4-dihydroxyphenyl)propanoate

C9H11NO4 (197.0688046)