2265PathwayIsoleucine DegradationThe degradation of isoleucine starts either in the mitochondria or the cytosol. L-isoleucine reacts with 2-oxoglutarate through a branch-chain amino acid aminotransferase resulting in the release of L-glutamate and 3-methyl-2-oxopentanoate. The latter compound reacts with 2-oxoisovalerate carboxy-lyase resulting in the release of carbon dioxide and methylbutanal. Methylbutanal can then be turned into methylbutanol through a alcohol dehydrogenaseMetabolicPW002491CenterPathwayVisualizationContext277719462420#000099PathwayVisualization22482265Isoleucine DegradationThe degradation of isoleucine starts either in the mitochondria or the cytosol. L-isoleucine reacts with 2-oxoglutarate through a branch-chain amino acid aminotransferase resulting in the release of L-glutamate and 3-methyl-2-oxopentanoate. The latter compound reacts with 2-oxoisovalerate carboxy-lyase resulting in the release of carbon dioxide and methylbutanal. Methylbutanal can then be turned into methylbutanol through a alcohol dehydrogenaseMetabolic18528310989420Dickinson JR: Pathways of leucine and valine catabolism in yeast. Methods Enzymol. 2000;324:80-92.2265Pathway528410753893Dickinson JR, Harrison SJ, Dickinson JA, Hewlins MJ: An investigation of the metabolism of isoleucine to active Amyl alcohol in Saccharomyces cerevisiae. J Biol Chem. 2000 Apr 14;275(15):10937-42.2265Pathway528512499363Dickinson JR, Salgado LE, Hewlins MJ: The catabolism of amino acids to long chain and complex alcohols in Saccharomyces cerevisiae. J Biol Chem. 2003 Mar 7;278(10):8028-34. doi: 10.1074/jbc.M211914200. Epub 2002 Dec 23.2265Pathway52868702755Eden A, Simchen G, Benvenisty N: Two yeast homologs of ECA39, a target for c-Myc regulation, code for cytosolic and mitochondrial branched-chain amino acid aminotransferases. J Biol Chem. 1996 Aug 23;271(34):20242-5.2265Pathway52878798704Kispal G, Steiner H, Court DA, Rolinski B, Lill R: Mitochondrial and cytosolic branched-chain amino acid transaminases from yeast, homologs of the myc oncogene-regulated Eca39 protein. J Biol Chem. 1996 Oct 4;271(40):24458-64.2265Pathway528810989445Prohl C, Kispal G, Lill R: Branched-chain-amino-acid transaminases of yeast Saccharomyces cerevisiae. Methods Enzymol. 2000;324:365-75.2265Pathway52899546164ter Schure EG, Flikweert MT, van Dijken JP, Pronk JT, Verrips CT: Pyruvate decarboxylase catalyzes decarboxylation of branched-chain 2-oxo acids but is not essential for fusel alcohol production by Saccharomyces cerevisiae. Appl Environ Microbiol. 1998 Apr;64(4):1303-7.2265Pathway1CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001823NeuronCL:00005401Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote18Saccharomyces cerevisiae4932EukaryoteYeast23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat24Solanum lycopersicum4081EukaryoteTomato21Xenopus laevis8355EukaryoteAfrican clawed frog4Arabidopsis thaliana3702EukaryoteThale cress49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia410Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria25Escherichia coli (strain K12)83333Prokaryote19Schizosaccharomyces pombe4896Eukaryote29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast7Chlamydomonas reinhardtii3055Eukaryote5CytoplasmGO:000573731Periplasmic SpaceGO:00056201CytosolGO:00058292MitochondrionGO:00057393Mitochondrial MatrixGO:000575913Endoplasmic ReticulumGO:000578324Mitochondrial Intermembrane SpaceGO:00057584PeroxisomeGO:000577710Cell MembraneGO:000588635ChloroplastGO:00095076LysosomeGO:000576411Extracellular SpaceGO:00056157Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574314Mitochondrial Outer MembraneGO:000574136MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032532Inner MembraneGO:007025825Golgi apparatusGO:000579416Lysosomal LumenGO:00432021LiverBTO:00007597299MuscleBTO:00008871411824BrainBTO:0000142891628StomachBTO:0001307155268Blood VesselBTO:000110274114Adrenal MedullaBTO:00000497188511PW_BS000008107313PW_BS00010710813PW_BS0001081873118PW_BS000024188118PW_BS000024315123PW_BS0000243183123PW_BS0000241115121PW_BS000111122551PW_BS0001221355171PW_BS0001352111PW_BS0000023211PW_BS0000034311PW_BS000004181311PW_BS000018311511PW_BS000031422411PW_BS0000425411PW_BS000005509516PW_BS000050261115PW_BS000026541315PW_BS000054103331PW_BS000103117131PW_BS0001171181171PW_BS0001181321121PW_BS0001321471241PW_BS0001471553241PW_BS0001551572241PW_BS0001571613181PW_BS0001611783211PW_BS00017885241011PW_BS000085222341PW_BS000024224241PW_BS000024151141PW_BS0001512253541PW_BS00002422014PW_BS0000241985181PW_BS0000242892491PW_BS000024253541PW_BS0000241333121PW_BS0001331122121PW_BS00011212915121PW_BS00012934524121PW_BS00002834695126PW_BS00002832711125PW_BS00002834713125PW_BS0000283344121PW_BS0000283683601PW_BS000028943PW_BS000094406351PW_BS000115124151PW_BS000124407251PW_BS0001154141551PW_BS0001154182451PW_BS000115408451PW_BS0001154239556PW_BS0001154241155PW_BS0001154251355PW_BS0001151203171PW_BS0001201192171PW_BS00011945015171PW_BS00011545424171PW_BS0001153744171PW_BS00005345895176PW_BS00011545911175PW_BS00011546013175PW_BS0001154793101PW_BS0001152991101PW_BS0000244812101PW_BS0001152975101PW_BS00002448924101PW_BS0001154824101PW_BS000115501361PW_BS000115388161PW_BS000112206261PW_BS000024205561PW_BS0000245062461PW_BS000115502461PW_BS00011511PW_BS00000114101PW_BS0000149611PW_BS0000091136121PW_BS000113105113PW_BS00010572513PW_BS000072711113PW_BS000071207661PW_BS0000242905491PW_BS0000243201123PW_BS000024126651PW_BS0001264436171PW_BS0001153016101PW_BS00002415111PW_BS00001549711PW_BS000049171211PW_BS000017221411PW_BS000022101711PW_BS0000107028511PW_BS000070100521PW_BS00010015924PW_BS00015916611PW_BS00016615284PW_BS000152101531PW_BS0001011632181PW_BS000163219314PW_BS0000242137181PW_BS00002421013181PW_BS00002421217181PW_BS0000241601181PW_BS00016017018PW_BS000170226441PW_BS00002416212181PW_BS0001621951318PW_BS0000242491341PW_BS0000241644PW_BS0001642811251PW_BS0000242851041PW_BS0000242863641PW_BS0000242875341PW_BS0000242273441PW_BS0000242231241PW_BS0000242941141PW_BS0000243081011PW_BS0000243221231PW_BS0000243125231PW_BS00002429341PW_BS00002413412121PW_BS0001343317121PW_BS0000281141112PW_BS00011413013121PW_BS000130310312PW_BS00002430412PW_BS000024383751PW_BS000100390761PW_BS0001123987171PW_BS0001133361121PW_BS000028109323PW_BS000109409115PW_BS0001153841251PW_BS0001001251351PW_BS0001251371117PW_BS00013712112171PW_BS00012113613171PW_BS0001364831110PW_BS0001154957101PW_BS00011548012101PW_BS00011530013101PW_BS000024208116PW_BS0000243911261PW_BS0001123951361PW_BS00011313121PW_BS000013204111PW_BS000020432511PW_BS0000431901118PW_BS0000242771218PW_BS0000243331212PW_BS00002834141121PW_BS00002835625121PW_BS000028412125PW_BS0001154192551PW_BS0001154461217PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS00011529111PW_BS0000292811611PW_BS0000286131PW_BS000006951721PW_BS0000951231751PW_BS00012314117191PW_BS0001412164181PW_BS00002429817101PW_BS00002433217121PW_BS000028429151PW_BS00011544717171PW_BS0001154641171PW_BS0001157081291PW_BS000512644171PW_BS000508112L-IsoleucineHMDB0000172Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. "BCAA" denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. BCAA are decreased in patients with liver disease, such as hepatitis, hepatic coma, cirrhosis, extrahepatic biliary atresia or portacaval shunt; aromatic amino acids (AAA)-tyrosine, tryptophan and phenylalanine, as well as methionine-are increased in these conditions. Valine, in particular, has been established as a useful supplemental therapy to the ailing liver. All the BCAA probably compete with AAA for absorption into the brain. Supplemental BCAA with vitamin B6 and zinc help normalize the BCAA:AAA ratio. The BCAA are not without side effects. Leucine alone, for example, exacerbates pellagra and can cause psychosis in pellagra patients by increasing excretion of niacin in the urine. Leucine may lower brain serotonin and dopamine. A dose of 3 g of isoleucine added to the niacin regime has cleared leucine-aggravated psychosis in schizophrenic patients. Isoleucine may have potential as an antipsychotic treatment. Leucine is more highly concentrated in foods than other amino acids. A cup of milk contains 800 mg of leucine and only 500 mg of isoleucine and valine. A cup of wheat germ has about 1.6 g of leucine and 1 g of isoleucine and valine. The ratio evens out in eggs and cheese. One egg and an ounce of most cheeses each contain about 400 mg of leucine and 400 mg of valine and isoleucine. The ratio of leucine to other BCAA is greatest in pork, where leucine is 7 to 8 g and the other BCAA together are only 3 to 4 g. (http://www.dcnutrition.com).73-32-5C00407630617191ILE6067DB00167CC[C@H](C)[C@H](N)C(O)=OC6H13NO2InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5-/m0/s1AGPKZVBTJJNPAG-WHFBIAKZSA-N(2S,3S)-2-amino-3-methylpentanoic acid131.1729131.094628665-0.062L-isoleucine00FDB012397(2s,3s)-2-amino-3-methylpentanoate;(2s,3s)-2-amino-3-methylpentanoic acid;(2s,3s)-2-amino-3-methyl-pentanoate;(2s,3s)-2-amino-3-methyl-pentanoic acid;(2s,3s)-a-amino-b-methyl-n-valerate;(2s,3s)-a-amino-b-methyl-n-valeric acid;(2s,3s)-a-amino-b-methylvalerate;(2s,3s)-a-amino-b-methylvaleric acid;(2s,3s)-alph-amino-beta-methylvalerate;(2s,3s)-alph-amino-beta-methylvaleric acid;(2s,3s)-alpha-amino-beta-merthyl-n-valerate;(2s,3s)-alpha-amino-beta-merthyl-n-valeric acid;(2s,3s)-alpha-amino-beta-merthylvalerate;(2s,3s)-alpha-amino-beta-merthylvaleric acid;(2s,3s)-alpha-amino-beta-methyl-n-valerate;(2s,3s)-alpha-amino-beta-methyl-n-valeric acid;(2s,3s)-alpha-amino-beta-methylvalerate;(2s,3s)-alpha-amino-beta-methylvaleric acid;(s)-isoleucine;(s,s)-isoleucine;2-amino-3-methylpentanoate;2-amino-3-methylpentanoic acid;2-amino-3-methylvalerate;2-amino-3-methylvaleric acid;2s,3s-isoleucine;Erythro-l-isoleucine;Ile;Iso-leucine;Isoleucine;L-(+)-isoleucine;L-ile;[s-(r*,r*)]-2-amino-3-methylpentanoate;[s-(r*,r*)]-2-amino-3-methylpentanoic acid;Alpha-amino-beta-methylvaleric acid;I;A-amino-b-methylvalerate;A-amino-b-methylvaleric acid;Alpha-amino-beta-methylvalerate;α-amino-β-methylvalerate;α-amino-β-methylvaleric acidPW_C000112Ile172485656107565710871471877148188425423154256231879183111121546122124104135134Oxoglutaric acidHMDB0000208Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acidPW_C000134AKG152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422011863198126812897705425377135133774811117752311277746129779673457797034677976327779843477842533480018368806941351131629411997240612002212412008440712017412212055241412081441812098940812114642312115242412116042512275712012283111912318645012339945412355437412371845812372445912373246012535747912540029912545548112553329712580048912592948212690050112694038812699320612706620512725550612738850295L-Glutamic acidHMDB0000148Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeurePW_C000095Glu1624436581191138416414969911054214485014562614625453231115344113541511754391185565132563110756321085859105600614760711576191946531856838187684418870927270937171652057182207751422475181518208225837322011792198118551611200422212621311268328912697290423483154234931842845320770202537733213377525112779713467797732777981347782913458064913512002312412004012212008640712034740612069212612081641812114742312115342412115742512283311912299712012329944312340145412371945812372545912372946012540129912541829712545748112566747912576930112580248912694138812699520612716250112725750642596(S)-3-methyl-2-oxopentanoate3S-Methyl-2-oxo-pentanoic acid, also known as (S)-3-methyl-2-oxopentanoate or (3S)-2-oxo-3-methyl-N-valeric acid, 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. 3S-Methyl-2-oxo-pentanoic acid is slightly soluble (in water) and a weakly acidic compound (based on its pKa). Outside of the human body, 3S-methyl-2-oxo-pentanoic acid can be found in a number of food items such as star fruit, common persimmon, oyster mushroom, and cashew nut. This makes 3S-methyl-2-oxo-pentanoic acid a potential biomarker for the consumption of these food products.CC[C@H](C)C(=O)C(O)=OC6H10O3InChI=1S/C6H10O3/c1-3-4(2)5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)/t4-/m0/s1JVQYSWDUAOAHFM-BYPYZUCNSA-N(3S)-3-methyl-2-oxopentanoic acid130.1418130.062994186-1.121(S)-omv0-1PW_C042596S3M2O7375188752322040034Hydrogen IonHMDB0059597Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])C000801038153781010[H+]HInChI=1S/p+1GPRLSGONYQIRFK-UHFFFAOYSA-Nhydron1.00791.0078250320hydron10H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+2154670875315788318483111621463261464542231492780174250224254424547104576184694705241103532711153531125626108563910756991005720105574211759631476037155607015760931616130159623216664831786601152669210168431886910187710016371682057191206745321974542207472222752521375322107558212757216075901708195225821815182432268413162842022491391959155249119151641201528112181285122462861226628712521227132572231332529415330308423293154235431842401322424053124245432076912293771361337721013477372331778041147795513277990327779913477837934579929130800193688038731080388304807221199382312494823383110550388112855941132803901155373981155391181158563361162051091199734061201934071205491221205934091211704241211714251225694181226153841226871251227581201231831351232181371237424591237434601251414541251881211252731361253594791255504811257304831257362971258092991265174951267174891267664801268233001269025011272132081283085061283613911284303951316Carbon dioxideHMDB0001967Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-Nmethanedione44.009543.9898292440.630carbon dioxide00DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO2508121120444801350318640367731695208065113343163849174522551173144705283103532011157501085771101596810060261556078161647117866371076922190701716070351637061188716320573081987333213746122275302108215225822315191582491184927711908170124642261268829042626315435233187699429377122133771701327747033377739112777501297776334178077134784053567842733478941331792271308000836880675119807171359483638411329139111554912111995440612008912212015540712036441212055641412083341912092212412099140812128412512150538312274412012301144612319045012341845512348911812355637412385513612406339812534447912546029712551648112582449012587029912593148212628048012688750112705220612727750712733138812739050212708()-2-MethylbutanalHMDB0031526()-2-Methylbutanal, also known as 2-methylbutyraldehyde, belongs to the class of organic compounds known as short-chain aldehydes. These are an aldehyde with a chain length containing between 2 and 5 carbon atoms ()-2-Methylbutanal is soluble (in water) and an extremely weak acidic (essentially neutral) compound (based on its pKa) ()-2-Methylbutanal has been detected in multiple biofluids, such as feces and saliva. Within the cell, ()-2-methylbutanal is primarily located in the cytoplasm ()-2-Methylbutanal can be converted into 2-methylbutanal oxime ()-2-Methylbutanal has been found to be associated with several diseases known as nonalcoholic fatty liver disease and ulcerative colitis; ()-2-methylbutanal has also been linked to the inborn metabolic disorders including celiac disease. ()-2-Methylbutanal is a flavouring agent. \n\n()-2-Methylbutanal belongs to the family of Aldehydes. These are organic compounds containing the aldehyde functional group.96-17-3C022237284161827012CCC(C)C=OC5H10OInChI=1S/C5H10O/c1-3-5(2)4-6/h4-5H,3H2,1-2H3BYGQBDHUGHBGMD-UHFFFAOYSA-N2-methylbutanal86.132386.073164942-0.6102-methylbutanal00C02223(+/-)-2-methylbutanal;(+/-)-2-methylbutyraldehyde;(rs)-2-methylbutanal;2-methylbutyraldehydePW_C0127082Mibual1144NADHHMDB0001487NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).58-68-4C0000443915316908NADH388299DB00157NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1OC21H29N7O14P2InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BOPGDPNILDQYTO-NNYOXOHSSA-N[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]({[(2R,3S,4R,5R)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy})phosphinic acid665.441665.124771695-2.358NADH0-2FDB0226491,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotidePW_C001144NADH1434153349086481011152127551469542230492781172836293109948061848121848212849046495931516995524010353321115358112546612354791255593135569810057371085829141591514759451516027155607916163871647217867711176893160701118870991637172205719520674622228244226836022590862241180919811821216123202491300329813015300132552234240332242618315771071327712313377208134773713317765133677668334777003327770713077917113779863478000936880691119938221241105493881128549411583811811995540612017240712037812212098640812116242512124412612169342912181838312261638412274512012312744712313813612355137412373446012381444312424246412437139812518912112534547912553148112576229712580829912592648212651649512676748012688850112738550212809039012836239112842939512709(S)-2-Methyl-1-butanolHMDB0031527(S)-2-Methyl-1-butanol is found in fruits. (S)-2-Methyl-1-butanol is isolated from grapes, apples, tomatoes etc.
(S)-2-Methyl-1-butanol belongs to the family of Primary Alcohols. These are compounds comprising the primary alcohol functional group, with the general strucuture RCOH (R=alkyl, aryl).137-32-68723489458398CCC(C)COC5H12OInChI=1S/C5H12O/c1-3-5(2)4-6/h5-6H,3-4H2,1-2H3QPRQEDXDYOZYLA-UHFFFAOYSA-N2-methylbutan-1-ol88.148288.088815006-0.3212-methyl-1-butanol00(+/-)-2-methyl-1-butanol;(1)-2-methylbutan-1-ol;(s)-(-)-2-methyl-1-butanol;(s)-2-methyl-1-butanol;(s)-2-methylbutan-1-ol;2-methyl butanol-1;2-methyl-(.+/-.)-1-butanol;2-methyl-(2s)-1-butanol;2-methyl-(s)-1-butanol;2-methyl-1-butanol;2-methyl-n-butanol;2-methylbutan-1-ol;2-methylbutanol;2-methylbutyl alcohol;Active amyl alcohol;Active primary amyl alcohol;Ch3ch2ch(ch3)ch2oh;D-2-methyl-1-butanol;Dl-2-methyl-1-butanol, pract;Dl-2-methyl-1-butanol;Dl-sec-butyl carbinol;L-2-methyl-1-butanol;Methyl-2-butan-1-ol;Primary active amyl alcohol;Sec-butyl carbinol;Sec-butylcarbinolPW_C012709S2M1Bol721NADHMDB0000902NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).53-84-9C00003589315846NAD5682NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1OC21H28N7O14P2InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1BAWFJGJZGIEFAR-NNYOXOHSSA-O1-[(2R,3R,4S,5R)-5-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium664.433664.116946663-2.5981-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium1-1FDB0223093-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nadPW_C000721NAD1404150335386511011142113443127351466542229492779172835293107948071848131848192849026496031516795523810353341115360112546912354821255590135561011856961005738108582714159121475942151602415560721576076161638516469178677211768901607012188709716371742057197206740519874592228241226835922590852241181921612322249130062981301830013256223424043224261931577104132771201337720913477370331776503367766733477702332777091307791511377983347784063568000636880690119938251241105523881127501661128539411992912211995240612017140712083441912098440812115942512124212612125942912181738312261438412274212012313044712314113612341945512354937412373146012381244312382946412437039812518712112531929712534247912553048112580629912582549012592448212651549512676548012688550112727850712738350212808939012836039112842839510936Branched-chain-amino-acid aminotransferase, mitochondrialP38891Involved in the biosynthesis of the branched chain amino acids leucine, isoleucine, and valine. Catalyzes the formation of methionine from 2-keto-4-methylthiobutyrate (KMTB) in the methionine salvage pathway primarily using branched chain amino acids (leucine, isoleucine, and valine) as the amino donors. Appears to be involved in the regulation of the transition from G1 to S phase in the cell cycle.BAT1292.6.1.42784016310937Branched-chain-amino-acid aminotransferase, cytosolicP47176
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. Catalyzes the formation of methionine from 2-keto-4-methylthiobutyrate (KMTB) in the methionine salvage pathway primarily using branched chain amino acids (leucine, isoleucine, and valine) as well as lysine and proline as the amino donors. Involved in cell cycle regulation.
BAT2292.6.1.42784116010761Pyruvate decarboxylase isozyme 1P06169
Major of three pyruvate decarboxylases (PDC1, PDC5, PDC6) implicated in the nonoxidative conversion of pyruvate to acetaldehyde and carbon dioxide during alcoholic fermentation. Most of the produced acetaldehyde is subsequently reduced to ethanol, but some is required for cytosolic acetyl-CoA production for biosynthetic pathways. The enzyme is also one of five 2-oxo acid decarboxylases (PDC1, PDC5, PDC6, ARO10, and THI3) able to decarboxylate more complex 2-oxo acids (alpha-ketoacids) than pyruvate, which seem mainly involved in amino acid catabolism. Here the enzyme catalyzes the decarboxylation of amino acids, which, in a first step, have been transaminated to the corresponding 2-oxo acids. In a third step, the resulting aldehydes are reduced to alcohols, collectively referred to as fusel oils or alcohols. Its preferred substrates are the transaminated amino acids valine, isoleucine, phenylalanine, and tryptophan, whereas leucine is no substrate. In a side-reaction the carbanionic intermediate (or active aldehyde) generated by decarboxylation or by activation of an aldehyde can react with an aldehyde via condensation (or carboligation) yielding a 2-hydroxy ketone, collectively called acyloins.
PDC1294.1.1.-; 4.1.1.1775216013708570810762pyruvate decarboxylase 2P16467
Second most abundant of three pyruvate decarboxylases (PDC1, PDC5, PDC6) implicated in the nonoxidative conversion of pyruvate to acetaldehyde and carbon dioxide during alcoholic fermentation. Most of the produced acetaldehyde is subsequently reduced to ethanol, but some is required for cytosolic acetyl-CoA production for biosynthetic pathways. The enzyme is also one of five 2-oxo acid decarboxylases (PDC1, PDC5, PDC6, ARO10, and THI3) able to decarboxylate more complex 2-oxo acids (alpha-keto-acids) than pyruvate, which seem mainly involved in amino acid catabolism. Here the enzyme catalyzes the decarboxylation of amino acids, which, in a first step, have been transaminated to the corresponding 2-oxo acids. In a third step, the resulting aldehydes are reduced to alcohols, collectively referred to as fusel oils or alcohols. Its preferred substrates are the transaminated amino acids valine, isoleucine, phenylalanine, and tryptophan, whereas leucine is no substrate. In a side-reaction the carbanionic intermediate (or active aldehyde) generated by decarboxylation or by activation of an aldehyde can react with an aldehyde via condensation (or carboligation) yielding a 2-hydroxy ketone, collectively called acyloins.
PDC5294.1.1.-; 4.1.1.1775416013609164413708670810763Pyruvate decarboxylase isozyme 3P26263
Minor of three pyruvate decarboxylases (PDC1, PDC5, PDC6) implicated in the nonoxidative conversion of pyruvate to acetaldehyde and carbon dioxide during alcoholic fermentation. Most of the produced acetaldehyde is subsequently reduced to ethanol, but some is required for cytosolic acetyl-CoA production for biosynthetic pathways. The enzyme is also one of five 2-oxo acid decarboxylases (PDC1, PDC5, PDC6, ARO10, and THI3) able to decarboxylate more complex 2-oxo acids (alpha-keto-acids) than pyruvate, which seem mainly involved in amino acid catabolism. Here the enzyme catalyzes the decarboxylation of amino acids, which, in a first step, have been transaminated to the corresponding 2-oxo acids. In a third step, the resulting aldehydes are reduced to alcohols, collectively referred to as fusel oils or alcohols. Its preferred substrates are the transaminated amino acids valine, isoleucine, phenylalanine, and tryptophan, whereas leucine is no substrate. In a side-reaction the carbanionic intermediate (or active aldehyde) generated by decarboxylation or by activation of an aldehyde can react with an aldehyde via condensation (or carboligation) yielding a 2-hydroxy ketone, collectively called acyloins. The expression level of this protein in the presence of fermentable carbon sources is so low that it can not compensate for the other two pyruvate decarboxylases to sustain fermentation.
PDC6294.1.1.1775316013609264413708770810641alcohol dehydrogenase subunit IP25377
NADP-dependent alcohol dehydrogenase with a broad substrate specificity.
ADH7291.1.1.2775516013609364413708870810642alcohol dehydrogenase subunit 2Q04894
NADP-dependent alcohol dehydrogenase with a broad substrate specificity.
ADH6291.1.1.277561601360946441370897084713Branched-chain-amino-acid aminotransferase, mitochondrial18PW_P00471311894109364714Branched-chain-amino-acid aminotransferase, cytosolic18PW_P00471411895109374408pyruvate decarboxylase18PW_P0044081149510761114961076211497107634337alcohol dehydrogenase18PW_P004337113981064111399106426977falsePW_R006977Right287361121Compoundfalse287371341Compoundfalse28738951Compoundfalse28739425961Compoundfalse695447136978falsePW_R006978Right287401121Compoundfalse28741400341Compoundfalse2874213161Compoundfalse28743425961Compoundfalse695547146979falsePW_R006979Right28744425961Compoundfalse28745400341Compoundfalse28746127081Compoundfalse2874713161Compoundfalse695644086980falsePW_R006980Right28748127081Compoundfalse2874911441Compoundfalse28750400341Compoundfalse28751127091Compoundfalse287527211Compoundfalse69574337561PW_T000561707425961Compound161188Right813151123false44348610regular100100813161343false54362610regular10011081317953false101862610regular10011081318425963false113847610regular10012081319425961883false113891810regular100120813201123false39392710regular100100813214003455false504109210regular787881322131652false1004109810regular7878813234003455false1249104010regular787881324127083false172892110regular10011081325131652false1594104110regular787881326114460false1915106110regular5030813274003455false1960112210regular787881328127093false1729147610regular1001108132972159false1924142610regular503038651109361632false7735038subunitregular1507038652109371602false7189418subunitregular1507038653107611602false14539038subunitregular1507038654107621602false12989038subunitregular1507038655107631602false13789438subunitregular1507038656106411602false177612668subunitregular1507038657106421602false170112318subunitregular1507031461471322483797538651314624714224837976386523146344082248379773865337978386543797938655314644337224837980386563798138657116735M543 536 C573 536 743 538 773 538 5false18116736M643 681 C643 540 743 538 773 538 5false18116737M1018 681 C1015 540 953 538 923 538 5false18trueM 259.94685504416486 334.261556296296 L 245 333 L 251.38088772118584 346.5751343230783false116738M1138 536 C1108 536 953 538 923 538 5false18trueM 259.94685504416486 334.261556296296 L 245 333 L 251.38088772118584 346.5751343230783false116739M1188 596 C1188 626 1188 641 1188 671 83false18116740M1188 918 C1188 888 1188 701 1188 671 83false18trueM 259.94685504416486 436.261556296296 L 245 435 L 251.38088772118584 448.5751343230783false116741M493 977 C523 977 688 976 718 976 5false18116742M582 1131 C580 981 688 976 718 976 5false18116743M999 1132 C999 982 898 976 868 976 5false18trueM 259.94685504416486 816.261556296296 L 245 815 L 251.38088772118584 828.5751343230784false116744M1138 978 C1108 978 898 976 868 976 5false18trueM 259.94685504416486 816.261556296296 L 245 815 L 251.38088772118584 828.5751343230784false116745M1238 978 C1268 978 1348 978 1378 978 5false18116746M1327 1079 C1333 982 1348 978 1378 978 5false18116747M1728 976 C1698 976 1558 978 1528 978 5false18trueM 259.94685504416486 789.261556296296 L 245 788 L 251.38088772118584 801.5751343230784false116748M1589 1075 C1582 985 1558 978 1528 978 5false18trueM 259.94685504416486 789.261556296296 L 245 788 L 251.38088772118584 801.5751343230784false116749M1778 1031 C1778 1061 1776 1201 1776 1231 5false18116750M1906 1071 C1775 1074 1776 1127 1776 1226 5false18116751M1960 1161 C1780 1165 1778 1165 1776 1231 5false18116752M1779 1476 C1779 1446 1776 1331 1776 1301 5false18trueM 1097.9468550441647 1038.261556296296 L 1083 1037 L 1089.380887721186 1050.5751343230784false116753M1949 1426 C1782 1423 1776 1417 1776 1301 5false18trueM 1097.9468550441647 1038.261556296296 L 1083 1037 L 1089.380887721186 1050.5751343230784false23955224869779234981315116735Left9235081316116736Left9235181317116737Right9235281318116738Right2375469543146123956224869789235381320116741Left9235481321116742Left9235581322116743Right9235681319116744Right2375569553146223957224869799235781319116745Left9235881323116746Left9235981324116747Right9236081325116748Right2375669563146323958224869809236181324116749Left9236281326116750Left9236381327116751Left9236481328116752Right9236581329116753Right2375769573146410635612248258181318116739Left258281319116740Right154219593651.01.0902904844974380M125 225 C125 175 175 125 225 125 C817 125 1586 125 2178 125 C2228 125 2278 175 2278 225 C2278 678 2278 1268 2278 1721 C2278 1771 2228 1821 2178 1821 C1586 1821 817 1821 225 1821 C175 1821 125 1771 125 1721 C125 1268 125 678 125 225 1true62153.01696.04381M276 388 C276 338 326 288 376 288 C873 288 1520 288 2017 288 C2067 288 2117 338 2117 388 C2117 750 2117 1219 2117 1581 C2117 1631 2067 1681 2017 1681 C1520 1681 873 1681 376 1681 C326 1681 276 1631 276 1581 C276 1219 276 750 276 388 1true61841.01393.07331534632375398133778640#FFEBEB4962388