Reaction Process: Reactome:R-BTA-1614603
Cysteine formation from homocysteine related metabolites
find 7 related metabolites which is associated with chemical reaction(pathway) Cysteine formation from homocysteine
H2O + L-Cystathionine ⟶ 2OBUTA + L-Cys + ammonia
L-Cystathionine
Cystathionine is a dipeptide formed by serine and homocysteine. Cystathioninuria is a prominent manifestation of vitamin-B6 deficiency. The transsulfuration of methionine yields homocysteine, which combines with serine to form cystathionine, the proximate precursor of cysteine through the enzymatic activity of cystathionase. In conditions in which cystathionine gamma-synthase or cystathionase is deficient, for example, there is cystathioninuria. Although cystathionine has not been detected in normal human serum or plasma by most conventional methods, gas chromatographic/mass spectrometric methodology detected a mean concentration of cystathionine in normal human serum of 140 nM, with a range of 65 to 301 nM. Cystathionine concentrations in CSF have been 10, 1, and 0.5 uM, and "not detected". Only traces (i.e., <1 uM) of cystathionine are present in normal CSF.587. Gamma-cystathionase deficiency (also known as Cystathioninuria), which is an autosomal recessive disorder (NIH: 2428), provided the first instance in which, in a human, the major biochemical abnormality due to a defined enzyme defect was clearly shown to be alleviated by administration of large doses of pyridoxine. The response in gamma-cystathionase-deficient patients is not attributable to correction of a preexisting deficiency of this vitamin (OMMBID, Chap. 88). Isolated from Phallus impudicus (common stinkhorn) CONFIDENCE standard compound; INTERNAL_ID 146 KEIO_ID C019; [MS2] KO008910 KEIO_ID C047 KEIO_ID C019 Acquisition and generation of the data is financially supported in part by CREST/JST. CONFIDENCE standard compound; ML_ID 30 L-Cystathionine is a nonprotein thioether and is a key amino acid associated with the metabolic state of sulfur-containing amino acids. L-Cystathionine protects against Homocysteine-induced mitochondria-dependent apoptosis of vascular endothelial cells (HUVECs). L-Cystathionine plays an important role in cardiovascular protection[1][2]. L-Cystathionine is a nonprotein thioether and is a key amino acid associated with the metabolic state of sulfur-containing amino acids. L-Cystathionine protects against Homocysteine-induced mitochondria-dependent apoptosis of vascular endothelial cells (HUVECs). L-Cystathionine plays an important role in cardiovascular protection[1][2].
Alpha-ketobutyrate
3-methyl pyruvic acid, also known as alpha-ketobutyric acid or 2-oxobutyric acid, belongs to short-chain keto acids and derivatives class of compounds. Those are keto acids with an alkyl chain the contains less than 6 carbon atoms. Thus, 3-methyl pyruvic acid is considered to be a fatty acid lipid molecule. 3-methyl pyruvic acid is soluble (in water) and a weakly acidic compound (based on its pKa). 3-methyl pyruvic acid can be found in a number of food items such as pepper (c. baccatum), triticale, european plum, and black walnut, which makes 3-methyl pyruvic acid a potential biomarker for the consumption of these food products. 3-methyl pyruvic acid can be found primarily in blood, cerebrospinal fluid (CSF), saliva, and urine. 3-methyl pyruvic acid exists in all living species, ranging from bacteria to humans. In humans, 3-methyl pyruvic acid is involved in several metabolic pathways, some of which include methionine metabolism, homocysteine degradation, threonine and 2-oxobutanoate degradation, and propanoate metabolism. 3-methyl pyruvic acid is also involved in several metabolic disorders, some of which include dimethylglycine dehydrogenase deficiency, methylenetetrahydrofolate reductase deficiency (MTHFRD), s-adenosylhomocysteine (SAH) hydrolase deficiency, and hyperglycinemia, non-ketotic. 2-Ketobutyric acid, also known as alpha-ketobutyrate or 2-oxobutyrate, belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms. 2-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle. More specifically, 2-ketobutyric acid is a product of the lysis of cystathionine. 2-Oxobutanoic acid is a product in the enzymatic cleavage of cystathionine.
Water
Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71\\% of Earths surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the bodys solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the suns energy to split off waters hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the suns energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia). Water, also known as purified water or dihydrogen oxide, 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. Water can be found in a number of food items such as caraway, oxheart cabbage, alaska wild rhubarb, and japanese walnut, which makes water a potential biomarker for the consumption of these food products. Water can be found primarily in most biofluids, including ascites Fluid, blood, cerebrospinal fluid (CSF), and lymph, as well as throughout all human tissues. Water exists in all living species, ranging from bacteria to humans. In humans, water is involved in several metabolic pathways, some of which include cardiolipin biosynthesis CL(20:4(5Z,8Z,11Z,14Z)/18:0/20:4(5Z,8Z,11Z,14Z)/18:2(9Z,12Z)), cardiolipin biosynthesis cl(i-13:0/i-15:0/i-20:0/i-24:0), cardiolipin biosynthesis CL(18:0/18:0/20:4(5Z,8Z,11Z,14Z)/22:5(7Z,10Z,13Z,16Z,19Z)), and cardiolipin biosynthesis cl(a-13:0/i-18:0/i-13:0/i-19:0). Water is also involved in several metabolic disorders, some of which include de novo triacylglycerol biosynthesis tg(i-21:0/i-13:0/21:0), de novo triacylglycerol biosynthesis tg(22:0/20:0/i-20:0), de novo triacylglycerol biosynthesis tg(a-21:0/i-20:0/i-14:0), and de novo triacylglycerol biosynthesis tg(i-21:0/a-17:0/i-12:0). Water is a drug which is used for diluting or dissolving drugs for intravenous, intramuscular or subcutaneous injection, according to instructions of the manufacturer of the drug to be administered [fda label]. Water plays an important role in the world economy. Approximately 70\\% of the freshwater used by humans goes to agriculture. Fishing in salt and fresh water bodies is a major source of food for many parts of the world. Much of long-distance trade of commodities (such as oil and natural gas) and manufactured products is transported by boats through seas, rivers, lakes, and canals. Large quantities of water, ice, and steam are used for cooling and heating, in industry and homes. Water is an excellent solvent for a wide variety of chemical substances; as such it is widely used in industrial processes, and in cooking and washing. Water is also central to many sports and other forms of entertainment, such as swimming, pleasure boating, boat racing, surfing, sport fishing, and diving .
ammonia
An azane that consists of a single nitrogen atom covelently bonded to three hydrogen atoms. Ammonia, also known as nh3 or ammonia solution, 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. Ammonia can be found in a number of food items such as rose hip, yardlong bean, cereals and cereal products, and ceylon cinnamon, which makes ammonia a potential biomarker for the consumption of these food products. Ammonia can be found primarily in blood, cellular cytoplasm, cerebrospinal fluid (CSF), and urine, as well as throughout all human tissues. Ammonia exists in all eukaryotes, ranging from yeast to humans. In humans, ammonia is involved in several metabolic pathways, some of which include glucose-alanine cycle, phenylalanine and tyrosine metabolism, homocysteine degradation, and d-arginine and d-ornithine metabolism. Ammonia is also involved in several metabolic disorders, some of which include ureidopropionase deficiency, hyperornithinemia-hyperammonemia-homocitrullinuria [hhh-syndrome], non ketotic hyperglycinemia, and beta-mercaptolactate-cysteine disulfiduria. Moreover, ammonia is found to be associated with 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-Methyl-crotonyl-glycinuria, citrullinemia type I, and short bowel syndrome. Ammonia is a non-carcinogenic (not listed by IARC) potentially toxic compound. Ammonia or azane is a compound of nitrogen and hydrogen with the formula NH3. The simplest pnictogen hydride, ammonia is a colourless gas with a characteristic pungent smell. It is a common nitrogenous waste, particularly among aquatic organisms, and it contributes significantly to the nutritional needs of terrestrial organisms by serving as a precursor to food and fertilizers. Ammonia, either directly or indirectly, is also a building block for the synthesis of many pharmaceutical products and is used in many commercial cleaning products . Acute Exposure: EYES: irrigate opened eyes for several minutes under running water. INGESTION: do not induce vomiting. Rinse mouth with water (never give anything by mouth to an unconscious person). Seek immediate medical advice. SKIN: should be treated immediately by rinsing the affected parts in cold running water for at least 15 minutes, followed by thorough washing with soap and water. If necessary, the person should shower and change contaminated clothing and shoes, and then must seek medical attention. INHALATION: supply fresh air. If required provide artificial respiration. (z)-n-coumaroyl-5-hydroxyanthranilic acid is a member of the class of compounds known as avenanthramides. Avenanthramides are a group of phenolic alkaloids consisting of conjugate of three phenylpropanoids (ferulic, caffeic, or p-coumaric acid) and anthranilic acid (z)-n-coumaroyl-5-hydroxyanthranilic acid is practically insoluble (in water) and a weakly acidic compound (based on its pKa). (z)-n-coumaroyl-5-hydroxyanthranilic acid can be found in cereals and cereal products and oat, which makes (z)-n-coumaroyl-5-hydroxyanthranilic acid a potential biomarker for the consumption of these food products.