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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:40 UTC
HMDB IDHMDB0000051
Secondary Accession Numbers
  • HMDB00051
Metabolite Identification
Common NameAmmonia
DescriptionAmmonia is a colourless alkaline gas and is one of the most abundant nitrogen-containing compounds in the atmosphere. It is an irritant with a characteristic pungent odor that is widely used in industry. Inasmuch as ammonia is highly soluble in water and, upon inhalation, is deposited in the upper airways, occupational exposures to ammonia have commonly been associated with sinusitis, upper airway irritation, and eye irritation. Acute exposures to high levels of ammonia have also been associated with diseases of the lower airways and interstitial lung. Small amounts of ammonia are naturally formed in nearly all tissues and organs of the vertebrate organism. Ammonia is both a neurotoxin and a metabotoxin. In fact, it is the most common endogenous neurotoxin. A neurotoxin is a compound that causes damage to neural tissue and neural cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Ammonia is recognized to be central in the pathogenesis of a brain condition known as hepatic encephalopathy, which arises from various liver diseases and leads to a build up ammonia in the blood (hyperammonemia). More than 40% of people with cirrhosis develop hepatic encephalopathy. Part of the neurotoxicity of ammonia arises from the fact that it easily crosses the blood-brain barrier and is absorbed and metabolized by the astrocytes, a population of cells in the brain that constitutes 30% of the cerebral cortex. Astrocytes use ammonia when synthesizing glutamine from glutamate. The increased levels of glutamine lead to an increase in osmotic pressure in the astrocytes, which become swollen. There is increased activity of the inhibitory gamma-aminobutyric acid (GABA) system, and the energy supply to other brain cells is decreased. This can be thought of as an example of brain edema. The source of the ammonia leading to hepatic encaphlopahy is not entirely clear. The gut produces ammonia, which is metabolized in the liver, and almost all organ systems are involved in ammonia metabolism. Colonic bacteria produce ammonia by splitting urea and other amino acids, however this does not fully explain hyperammonemia and hepatic encephalopathy. The alternative explanation is that hyperammonemia is the result of intestinal breakdown of amino acids, especially glutamine. The intestines have significant glutaminase activity, predominantly located in the enterocytes. On the other hand, intestinal tissues only have a little glutamine synthetase activity, making it a major glutamine-consuming organ. In addition to the intestine, the kidney is an important source of blood ammonia in patients with liver disease. Ammonia is also taken up by the muscle and brain in hepatic coma, and there is confirmation that ammonia is metabolized in muscle. Excessive formation of ammonia in the brains of Alzheimer's disease patients has also been demonstrated, and it has been shown that some Alzheimer's disease patients exhibit elevated blood ammonia concentrations. Ammonia is the most important natural modulator of lysosomal protein processing. Indeed, there is strong evidence for the involvement of aberrant lysosomal processing of beta-amyloid precursor protein (beta-APP) in the formation of amyloid deposits. Inflammatory processes and activation of microglia are widely believed to be implicated in the pathology of Alzheimer's disease. Ammonia is able to affect the characteristic functions of microglia, such as endocytosis, and cytokine production. Based on these facts, an ammonia-based hypothesis for Alzheimer's disease has been suggested (PMID: 17006913 , 16167195 , 15377862 , 15369278 ). Chronically high levels of ammonia in the blood are associated with nearly twenty different inborn errors of metabolism including: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methyl-crotonylglycinuria, argininemia, argininosuccinic aciduria, beta-ketothiolase deficiency, biotinidase deficiency, carbamoyl phosphate synthetase deficiency, carnitine-acylcarnitine translocase deficiency, citrullinemia type I, hyperinsulinism-hyperammonemia syndrome, hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, isovaleric aciduria, lysinuric protein intolerance, malonic aciduria, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, propionic acidemia, pyruvate carboxylase deficiency, and short chain acyl CoA dehydrogenase deficiency (SCAD deficiency). Many of these inborn errors of metabolism are associated with urea cycle disorders or impairment to amino acid metabolism. High levels of ammonia in the blood (hyperammonemia) lead to the activation of NMDA receptors in the brain. This results in the depletion of brain ATP, which in turn leads to release of glutamate. Ammonia also leads to the impairment of mitochondrial function and calcium homeostasis, thereby decreasing ATP synthesis. Excess ammonia also increases the formation of nitric oxide (NO), which in turn reduces the activity of glutamine synthetase, and thereby decreases the elimination of ammonia in the brain (PMID: 12020609 ). As a neurotoxin, ammonia predominantly affects astrocytes. Disturbed mitochondrial function and oxidative stress, factors implicated in the induction of the mitochondrial permeability transition, appear to be involved in the mechanism of ammonia neurotoxicity. Ammonia can also affect the glutamatergic and GABAergic neuronal systems, the two prevailing neuronal systems of the cortical structures. All of these effects can lead to irreversible brain damage, coma, and/or death. Infants with urea cycle disorders and hyperammonia initially exhibit vomiting and increasing lethargy. If untreated, seizures, hypotonia (poor muscle tone, floppiness), respiratory distress (respiratory alkalosis), and coma can occur. Adults with urea cycle disorders and hyperammonia will exhibit episodes of disorientation, confusion, slurred speech, unusual and extreme combativeness or agitation, stroke-like symptoms, lethargy, and delirium. Ammonia also has toxic effects when an individual is exposed to ammonia solutions. Acute exposure to high levels of ammonia in air may be irritating to skin, eyes, throat, and lungs and cause coughing and burns. Lung damage and death may occur after exposure to very high concentrations of ammonia. Swallowing concentrated solutions of ammonia can cause burns in the mouth, throat, and stomach. Splashing ammonia into eyes can cause burns and even blindness.
Structure
Thumb
Synonyms
ValueSource
[NH3]ChEBI
AmmoniacChEBI
AmmoniakChEBI
AmoniacoChEBI
NH3ChEBI
R-717ChEBI
Spirit OF hartshornChEBI
Ammonia anhydrousHMDB
Ammonia inhalantHMDB
Ammonia solution strongHMDB
Ammonia waterHMDB
Liquid ammoniaHMDB
Am-folHMDB
Ammonia (CONC 20% or greater)HMDB
Ammonia gasHMDB
Ammonia solutionHMDB
Ammonia solution strong (NF)HMDB
Ammonia water (JP15)HMDB
Ammoniacum gummiHMDB
Ammoniak kconzentrierterHMDB
AmmoniakgasHMDB
Ammonium ionHMDB
Anhydrous ammoniaHMDB
Aromatic ammonia vaporoleHMDB
AzaneHMDB
NH(3)HMDB
nitro-SilHMDB
Primaeres aminHMDB
Sekundaeres aminHMDB
Tertiaeres aminHMDB
Chemical FormulaH3N
Average Molecular Weight17.0305
Monoisotopic Molecular Weight17.026549101
IUPAC Nameammonia
Traditional Nameammonia
CAS Registry Number7664-41-7
SMILES
N
InChI Identifier
InChI=1S/H3N/h1H3
InChI KeyQGZKDVFQNNGYKY-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of inorganic compounds known as homogeneous other non-metal compounds. These are inorganic non-metallic compounds in which the largest atom belongs to the class of 'other non-metals'.
KingdomInorganic compounds
Super ClassHomogeneous non-metal compounds
ClassHomogeneous other non-metal compounds
Sub ClassNot Available
Direct ParentHomogeneous other non-metal compounds
Alternative ParentsNot Available
Substituents
  • Homogeneous other non metal
Molecular FrameworkNot Available
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Biological role:

Environmental role:

Industrial application:

Physical Properties
StateLiquid
Experimental Properties
PropertyValueReference
Melting Point-77.7 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility482 mg/mL at 24 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
logP-0.98ChemAxon
pKa (Strongest Basic)8.86ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count1ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area13.59 ŲChemAxon
Rotatable Bond Count0ChemAxon
Refractivity15.51 m³·mol⁻¹ChemAxon
Polarizability1.99 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-014i-9000000000-92ab2d6b6fd9cfb23ac7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-014i-9000000000-88ae09421d46f7dea1c5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-014i-9000000000-88ae09421d46f7dea1c5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-014i-9000000000-88ae09421d46f7dea1c5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-9000000000-5e750288766bc8c562ffView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-014i-9000000000-5e750288766bc8c562ffView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-014i-9000000000-5e750288766bc8c562ffView in MoNA
MSMass Spectrum (Electron Ionization)splash10-014i-9000000000-e0a6e51ead158714099bView in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Locations
  • All Tissues
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified<50 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified15-35 uMChildren (1-13 years old)Not Specified
Normal
details
BloodDetected and Quantified<60 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified<54 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified22-48 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified<50 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified15-45 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified21-50 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified<4.404 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<50 uMInfant (0-1 year old)MaleNormal details
BloodDetected and Quantified14-50 uMInfant (0-1 year old)BothNormal
    • Clinical and Labo...
details
BloodDetected and Quantified27.5 +/- 3.6 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified17-47 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified18 - 74 uMInfant (0-1 year old)MaleNormal details
BloodDetected and Quantified5.872-35.231 uMChildren (1 - 13 years old)BothNormal details
BloodDetected and Quantified13.000-42.000 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified29.0 (10.0-47.0) uMAdult (>18 years old)BothNormal
    • The Merck Manual,...
details
BloodDetected and Quantified40.0 (0 - 80.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified11-32 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified29.0 (13.0 - 46.0) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified35.0 (20.0 - 58.0) uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified29.0 (17.0 - 51.0) uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified1467.955 (763.336-2231.291) uMChildren (1 - 13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified9-33 uMAdolescent (13-18 years old)MaleNormal details
Cellular CytoplasmDetected and Quantified800 (700-900) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified11.9 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified2810.0 +/- 947.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified1900.0 +/- 350.0 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified2330.0 (724.0-3950.0) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified94 uMChildren (1-13 years old)MaleArgininosuccinic aciduria (ASL) details
    BloodDetected and Quantified62 uMInfant (0-1 year old)MaleArgininosuccinic aciduria (ASL) details
    BloodDetected and Quantified52.846 uMChildren (1-13 years old)FemaleDibasic Amino Aciduria I details
    BloodDetected and Quantified75-92 uMAdolescent (13-18 years old)Female
    3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
    details
    BloodDetected and Quantified15.6 +/- 13.5 uMChildren (1-13 years old)Female
    Pearson Syndrome
    details
    BloodDetected and Quantified60-120 uMChildren (1-13 years old)BothCutis laxa, autosomal recessive, type IIIA details
    BloodDetected and Quantified111-122 uMChildren (1-13 years old)FemaleMetabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration details
    BloodDetected and Quantified140-503 uMInfant (0-1 year old)Not AvailableMitochondrial trifunctional protein deficiency details
    BloodDetected and Quantified70 uMChildren (1-13 years old)Not AvailablePyruvate carboxylase deficiency details
    BloodDetected and Quantified1100.0 (200.0-2000.0) uMChildren (1-13 years old)BothArgininosuccinic aciduria (ASL)
      • MetaGene: Metabol...
    details
    BloodDetected and Quantified61-164 uMAdult (>18 years old)Both
    3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
    details
    BloodDetected and Quantified268 uMInfant (0-1 year old)Not AvailablePyruvate carboxylase deficiency details
    BloodDetected and Quantified40-145 uMChildren (1-13 years old)Both
    3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
    details
    BloodDetected and Quantified234 uMChildren (1-13 years old)FemaleCarnitine palmitoyltransferase deficiency I details
    BloodDetected and Quantified82-209 uMChildren (1-13 years old)BothInfantile Liver Failure Syndrome 2 details
    BloodDetected and Quantified65-145 uMChildren (1-13 years old)FemaleCerebral creatine deficiency syndrome 2 details
    BloodDetected and Quantified66-86 uMInfant (0-1 year old)FemaleInfantile Liver Failure Syndrome 2 details
    BloodDetected and Quantified138 uMInfant (0-1 year old)FemaleMetabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration details
    BloodDetected and Quantified338 uMInfant (0-1 year old)FemaleLong-chain Fatty Acids, Defect in Transport of details
    BloodDetected and Quantified110-270 uMNewborn (0-30 days old)MaleNarp Syndrome details
    BloodDetected and Quantified25-30 uMChildren (1-13 years old)BothPhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
    BloodDetected and Quantified146 uMNewborn (0-30 days old)MalePhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
    BloodDetected and Quantified1.703-14.0336 uMAdult (>18 years old)BothCitrullinemia type II, adult-onset details
    BloodDetected and Quantified49 uMInfant (0-1 year old)Male
    Sulfite oxidase deficiency
      • Clinical and Labo...
    details
    BloodDetected and Quantified13446.464-63415.637 uMChildren (1 - 13 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    BloodDetected and Quantified139 - 299 uMInfant (0-1 year old)MaleFumaric aciduria details
    BloodDetected and Quantified231(96-780) uMAdult (>18 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    BloodDetected and Quantified27-246 uMChildren (1-13 years old)Male
    N-acetylglutamate synthetase deficiency
    details
    BloodDetected and Quantified27.0104-572.502 uMChildren (1 - 13 years old)BothCitrullinemia Type I details
    BloodDetected and Quantified85-835 uMInfant (0-1 year old)Both
    3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
    details
    BloodDetected and Quantified105-500 uMNewborn (0-30 days old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    BloodDetected and Quantified44-84 uMInfant (0-1 year old)Male3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) details
    BloodDetected and Quantified63 uMNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
    BloodDetected and Quantified50.4 +/- 17.0 uMAdult (>18 years old)BothShort bowel syndrome details
    BloodDetected and Quantified>700 uMAdolescent (13-18 years old)MaleMitochondrial complex I deficiency due to ACAD9 deficiency details
    BloodDetected and Quantified252.488 +/- 46.387 uMAdult (>18 years old)Not Specified
    adult-type citrullinemia
    details
    BloodDetected and Quantified70.462 +/- 22.313 uMAdult (>18 years old)Not Specified
    adult-type citrullinemia
    details
    BloodDetected and Quantified52.5 (25.0-80.0) uMAdult (>18 years old)Both3-methyl-crotonyl-glycinuria
      • MetaGene: Metabol...
    details
    BloodDetected and Quantified200.0 (100.0-300.0) uMChildren (1-13 years old)Both3-Methyl-Crotonyl-Glycinuria
      • MetaGene: Metabol...
    details
    Associated Disorders and Diseases
    Disease References
    3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
    1. Muroi J, Yorifuji T, Uematsu A, Shigematsu Y, Onigata K, Maruyama H, Nobutoki T, Kitamura A, Nakahata T: Molecular and clinical analysis of Japanese patients with 3-hydroxy-3-methylglutaryl CoA lyase (HL) deficiency. Hum Genet. 2000 Oct;107(4):320-6. [PubMed:11129331 ]
    2. Grunert SC, Schlatter SM, Schmitt RN, Gemperle-Britschgi C, Mrazova L, Balci MC, Bischof F, Coker M, Das AM, Demirkol M, de Vries M, Gokcay G, Haberle J, Ucar SK, Lotz-Havla AS, Lucke T, Roland D, Rutsch F, Santer R, Schlune A, Staufner C, Schwab KO, Mitchell GA, Sass JO: 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: Clinical presentation and outcome in a series of 37 patients. Mol Genet Metab. 2017 Jul;121(3):206-215. doi: 10.1016/j.ymgme.2017.05.014. Epub 2017 May 22. [PubMed:28583327 ]
    3. Ozand PT, al Aqeel A, Gascon G, Brismar J, Thomas E, Gleispach H: 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency in Saudi Arabia. J Inherit Metab Dis. 1991;14(2):174-88. [PubMed:1886403 ]
    4. Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
    Argininosuccinic aciduria
    1. Kleijer WJ, Garritsen VH, Linnebank M, Mooyer P, Huijmans JG, Mustonen A, Simola KO, Arslan-Kirchner M, Battini R, Briones P, Cardo E, Mandel H, Tschiedel E, Wanders RJ, Koch HG: Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families. J Inherit Metab Dis. 2002 Sep;25(5):399-410. [PubMed:12408190 ]
    2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    3-Methyl-crotonyl-glycinuria
    1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    Carnitine palmitoyltransferase I deficiency
    1. Olpin SE, Allen J, Bonham JR, Clark S, Clayton PT, Calvin J, Downing M, Ives K, Jones S, Manning NJ, Pollitt RJ, Standing SJ, Tanner MS: Features of carnitine palmitoyltransferase type I deficiency. J Inherit Metab Dis. 2001 Feb;24(1):35-42. [PubMed:11286380 ]
    Citrullinemia type I
    1. Kose E, Unal O, Bulbul S, Gunduz M, Haberle J, Arslan N: Identification of three novel mutations in fourteen patients with citrullinemia type 1. Clin Biochem. 2017 Aug;50(12):686-689. doi: 10.1016/j.clinbiochem.2017.01.011. Epub 2017 Jan 27. [PubMed:28132756 ]
    Fumarase deficiency
    1. Bastug O, Kardas F, Ozturk MA, Halis H, Memur S, Korkmaz L, Tag Z, Gunes T: A rare cause of opistotonus; fumaric aciduria: The first case presentation in Turkey. Turk Pediatri Ars. 2014 Mar 1;49(1):74-6. doi: 10.5152/tpa.2014.442. eCollection 2014 Mar. [PubMed:26078636 ]
    Short bowel syndrome
    1. Pita AM, Wakabayashi Y, Fernandez-Bustos MA, Virgili N, Riudor E, Soler J, Farriol M: Plasma urea-cycle-related amino acids, ammonium levels, and urinary orotic acid excretion in short-bowel patients managed with an oral diet. Clin Nutr. 2003 Feb;22(1):93-8. [PubMed:12553956 ]
    Pyruvate carboxylase deficiency
    1. Habarou F, Brassier A, Rio M, Chretien D, Monnot S, Barbier V, Barouki R, Bonnefont JP, Boddaert N, Chadefaux-Vekemans B, Le Moyec L, Bastin J, Ottolenghi C, de Lonlay P: Pyruvate carboxylase deficiency: An underestimated cause of lactic acidosis. Mol Genet Metab Rep. 2014 Nov 28;2:25-31. doi: 10.1016/j.ymgmr.2014.11.001. eCollection 2015 Mar. [PubMed:28649521 ]
    Sulfite oxidase deficiency, ISOLATED
    1. Choong T. et al. (2010). Clinical and Laboratory Barriers to the Timely Diagnosis of Sulphite Oxidase Deficiency. Proceedings of Singapore Healthcare, 19(2), 94-100.. Proceedings of Singapore Healthcare.
    N-acetylglutamate synthetase deficiency
    1. Schubiger G, Bachmann C, Barben P, Colombo JP, Tonz O, Schupbach D: N-acetylglutamate synthetase deficiency: diagnosis, management and follow-up of a rare disorder of ammonia detoxication. Eur J Pediatr. 1991 Mar;150(5):353-6. [PubMed:2044610 ]
    Hyperdibasic aminoaciduria I
    1. Whelan DT, Scriver CR: Hyperdibasicaminoaciduria: an inherited disorder of amino acid transport. Pediatr Res. 1968 Nov;2(6):525-34. [PubMed:5727921 ]
    Mitochondrial complex I deficiency due to ACAD9 deficiency
    1. He M, Rutledge SL, Kelly DR, Palmer CA, Murdoch G, Majumder N, Nicholls RD, Pei Z, Watkins PA, Vockley J: A new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiency. Am J Hum Genet. 2007 Jul;81(1):87-103. Epub 2007 Jun 4. [PubMed:17564966 ]
    3-Hydroxyacyl-CoA dehydrogenase deficiency
    1. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA: Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr. 2001 Mar;138(3):383-9. doi: 10.1067/mpd.2001.111818. [PubMed:11241047 ]
    2. Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
    Cerebral creatine deficiency syndrome 2
    1. Schulze A, Hess T, Wevers R, Mayatepek E, Bachert P, Marescau B, Knopp MV, De Deyn PP, Bremer HJ, Rating D: Creatine deficiency syndrome caused by guanidinoacetate methyltransferase deficiency: diagnostic tools for a new inborn error of metabolism. J Pediatr. 1997 Oct;131(4):626-31. [PubMed:9386672 ]
    Citrullinemia type II, adult-onset
    1. Yajima Y, Hirasawa T, Saheki T: Diurnal fluctuation of blood ammonia levels in adult-type citrullinemia. Tohoku J Exp Med. 1982 Jun;137(2):213-20. [PubMed:7202267 ]
    2. Komatsu M, Yazaki M, Tanaka N, Sano K, Hashimoto E, Takei Y, Song YZ, Tanaka E, Kiyosawa K, Saheki T, Aoyama T, Kobayashi K: Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease. J Hepatol. 2008 Nov;49(5):810-20. doi: 10.1016/j.jhep.2008.05.016. Epub 2008 Jun 10. [PubMed:18620775 ]
    Cutis laxa, autosomal recessive, type IIIA
    1. Baumgartner MR, Hu CA, Almashanu S, Steel G, Obie C, Aral B, Rabier D, Kamoun P, Saudubray JM, Valle D: Hyperammonemia with reduced ornithine, citrulline, arginine and proline: a new inborn error caused by a mutation in the gene encoding delta(1)-pyrroline-5-carboxylate synthase. Hum Mol Genet. 2000 Nov 22;9(19):2853-8. [PubMed:11092761 ]
    Infantile Liver Failure Syndrome 2
    1. Staufner C, Haack TB, Kopke MG, Straub BK, Kolker S, Thiel C, Freisinger P, Baric I, McKiernan PJ, Dikow N, Harting I, Beisse F, Burgard P, Kotzaeridou U, Lenz D, Kuhr J, Himbert U, Taylor RW, Distelmaier F, Vockley J, Ghaloul-Gonzalez L, Ozolek JA, Zschocke J, Kuster A, Dick A, Das AM, Wieland T, Terrile C, Strom TM, Meitinger T, Prokisch H, Hoffmann GF: Recurrent acute liver failure due to NBAS deficiency: phenotypic spectrum, disease mechanisms, and therapeutic concepts. J Inherit Metab Dis. 2016 Jan;39(1):3-16. doi: 10.1007/s10545-015-9896-7. Epub 2015 Nov 5. [PubMed:26541327 ]
    Long-chain Fatty Acids, Defect in Transport of
    1. Treem WR, Stanley CA, Finegold DN, Hale DE, Coates PM: Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts. N Engl J Med. 1988 Nov 17;319(20):1331-6. doi: 10.1056/NEJM198811173192006. [PubMed:3185635 ]
    Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration
    1. Lalani SR, Liu P, Rosenfeld JA, Watkin LB, Chiang T, Leduc MS, Zhu W, Ding Y, Pan S, Vetrini F, Miyake CY, Shinawi M, Gambin T, Eldomery MK, Akdemir ZH, Emrick L, Wilnai Y, Schelley S, Koenig MK, Memon N, Farach LS, Coe BP, Azamian M, Hernandez P, Zapata G, Jhangiani SN, Muzny DM, Lotze T, Clark G, Wilfong A, Northrup H, Adesina A, Bacino CA, Scaglia F, Bonnen PE, Crosson J, Duis J, Maegawa GH, Coman D, Inwood A, McGill J, Boerwinkle E, Graham B, Beaudet A, Eng CM, Hanchard NA, Xia F, Orange JS, Gibbs RA, Lupski JR, Yang Y: Recurrent Muscle Weakness with Rhabdomyolysis, Metabolic Crises, and Cardiac Arrhythmia Due to Bi-allelic TANGO2 Mutations. Am J Hum Genet. 2016 Feb 4;98(2):347-57. doi: 10.1016/j.ajhg.2015.12.008. Epub 2016 Jan 21. [PubMed:26805781 ]
    Mitochondrial trifunctional protein deficiency
    1. den Boer ME, Dionisi-Vici C, Chakrapani A, van Thuijl AO, Wanders RJ, Wijburg FA: Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement. J Pediatr. 2003 Jun;142(6):684-9. doi: 10.1067/mpd.2003.231. [PubMed:12838198 ]
    Myopathy, lactic acidosis, and sideroblastic anemia 1
    1. Parfait B, de Lonlay P, von Kleist-Retzow JC, Cormier-Daire V, Chretien D, Rotig A, Rabier D, Saudubray JM, Rustin P, Munnich A: The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia. Eur J Pediatr. 1999 Jan;158(1):55-8. [PubMed:9950309 ]
    Pearson Syndrome
    1. Crippa BL, Leon E, Calhoun A, Lowichik A, Pasquali M, Longo N: Biochemical abnormalities in Pearson syndrome. Am J Med Genet A. 2015 Mar;167A(3):621-8. doi: 10.1002/ajmg.a.36939. [PubMed:25691415 ]
    Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic
    1. Vieira P, Cameron J, Rahikkala E, Keski-Filppula R, Zhang LH, Santra S, Matthews A, Myllynen P, Nuutinen M, Moilanen JS, Rodenburg RJ, Rolfs A, Uusimaa J, van Karnebeek CDM: Novel homozygous PCK1 mutation causing cytosolic phosphoenolpyruvate carboxykinase deficiency presenting as childhood hypoglycemia, an abnormal pattern of urine metabolites and liver dysfunction. Mol Genet Metab. 2017 Apr;120(4):337-341. doi: 10.1016/j.ymgme.2017.02.003. Epub 2017 Feb 6. [PubMed:28216384 ]
    2. Santra S, Cameron JM, Shyr C, Zhang L, Drogemoller B, Ross CJ, Wasserman WW, Wevers RA, Rodenburg RJ, Gupte G, Preece MA, van Karnebeek CD: Cytosolic phosphoenolpyruvate carboxykinase deficiency presenting with acute liver failure following gastroenteritis. Mol Genet Metab. 2016 May;118(1):21-7. doi: 10.1016/j.ymgme.2016.03.001. Epub 2016 Mar 4. [PubMed:26971250 ]
    Associated OMIM IDs
    • 210200 (3-Methyl-crotonyl-glycinuria)
    • 207900 (Argininosuccinic aciduria)
    • 272300 (Sulfite oxidase deficiency, ISOLATED)
    • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    • 606812 (Fumarase deficiency)
    • 608300 (N-acetylglutamate synthetase deficiency)
    • 215700 (Citrullinemia type I)
    • 603471 (Citrullinemia type II, adult-onset)
    • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
    • 611126 (Mitochondrial complex I deficiency due to ACAD9 deficiency)
    • 222690 (Hyperdibasic aminoaciduria I)
    • 557000 (Pearson Syndrome)
    • 219150 (Cutis laxa, autosomal recessive, type IIIA)
    • 616878 (Metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration)
    • 609015 (Mitochondrial trifunctional protein deficiency)
    • 266150 (Pyruvate carboxylase deficiency)
    • 255120 (Carnitine palmitoyltransferase I deficiency)
    • 612736 (Cerebral creatine deficiency syndrome 2)
    • 616483 (Infantile Liver Failure Syndrome 2)
    • 603376 (Long-chain Fatty Acids, Defect in Transport of)
    • 600462 (Myopathy, lactic acidosis, and sideroblastic anemia 1)
    • 261680 (Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic)
    DrugBank IDNot Available
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB003908
    KNApSAcK IDC00007267
    Chemspider ID217
    KEGG Compound IDC00014
    BioCyc IDAMMONIA
    BiGG IDNot Available
    Wikipedia LinkAmmonia
    METLIN ID3189
    PubChem Compound222
    PDB IDNH3
    ChEBI ID16134
    References
    Synthesis ReferenceMohr, Rudolf. Ammonia separation from offgas obtained from melamine synthesis. U.S. (1971), 5 pp. CODEN: USXXAM US 3555784 19710119 CAN 77:50902 AN 1972:450902
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Yoshida Y, Higashi T, Nouso K, Nakatsukasa H, Nakamura SI, Watanabe A, Tsuji T: Effects of zinc deficiency/zinc supplementation on ammonia metabolism in patients with decompensated liver cirrhosis. Acta Med Okayama. 2001 Dec;55(6):349-55. [PubMed:11779097 ]
    2. Huizenga JR, Teelken AW, Tangerman A, de Jager AE, Gips CH, Jansen PL: Determination of ammonia in cerebrospinal fluid using the indophenol direct method. Mol Chem Neuropathol. 1998 Jun-Aug;34(2-3):169-77. [PubMed:10327416 ]
    3. Cohen BI: The significance of ammonia/gamma-aminobutyric acid (GABA) ratio for normality and liver disorders. Med Hypotheses. 2002 Dec;59(6):757-8. [PubMed:12445521 ]
    4. Kochar DK, Agarwal P, Kochar SK, Jain R, Rawat N, Pokharna RK, Kachhawa S, Srivastava T: Hepatocyte dysfunction and hepatic encephalopathy in Plasmodium falciparum malaria. QJM. 2003 Jul;96(7):505-12. [PubMed:12881593 ]
    5. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
    6. Cooper AJ: Role of glutamine in cerebral nitrogen metabolism and ammonia neurotoxicity. Ment Retard Dev Disabil Res Rev. 2001;7(4):280-6. [PubMed:11754523 ]
    7. Remer T: Influence of nutrition on acid-base balance--metabolic aspects. Eur J Nutr. 2001 Oct;40(5):214-20. [PubMed:11842946 ]
    8. Kaiho T, Tanaka T, Tsuchiya S, Yanagisawa S, Takeuchi O, Miura M, Saigusa N, Miyazaki M: Effect of the herbal medicine Dai-kenchu-to for serum ammonia in hepatectomized patients. Hepatogastroenterology. 2005 Jan-Feb;52(61):161-5. [PubMed:15783019 ]
    9. Nybo L, Dalsgaard MK, Steensberg A, Moller K, Secher NH: Cerebral ammonia uptake and accumulation during prolonged exercise in humans. J Physiol. 2005 Feb 15;563(Pt 1):285-90. Epub 2004 Dec 20. [PubMed:15611036 ]
    10. Huizenga JR, Vissink A, Kuipers EJ, Gips CH: Helicobacter pylori and ammonia concentrations of whole, parotid and submandibular/sublingual saliva. Clin Oral Investig. 1999 Jun;3(2):84-7. [PubMed:10803116 ]
    11. Satoh M, Yokoya S, Hachiya Y, Hachiya M, Fujisawa T, Hoshino K, Saji T: Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr. 2001 May;160(5):307-11. [PubMed:11388600 ]
    12. Suarez I, Bodega G, Fernandez B: Glutamine synthetase in brain: effect of ammonia. Neurochem Int. 2002 Aug-Sep;41(2-3):123-42. [PubMed:12020613 ]
    13. Helewski K, Kowalczyk-Ziomek G, Konecki J: [Ammonia and GABA-ergic neurotransmission in pathogenesis of hepatic encephalopathy]. Wiad Lek. 2003;56(11-12):560-3. [PubMed:15058165 ]
    14. Grasten SM, Juntunen KS, Poutanen KS, Gylling HK, Miettinen TA, Mykkanen HM: Rye bread improves bowel function and decreases the concentrations of some compounds that are putative colon cancer risk markers in middle-aged women and men. J Nutr. 2000 Sep;130(9):2215-21. [PubMed:10958815 ]
    15. Pita AM, Wakabayashi Y, Fernandez-Bustos MA, Virgili N, Riudor E, Soler J, Farriol M: Plasma urea-cycle-related amino acids, ammonium levels, and urinary orotic acid excretion in short-bowel patients managed with an oral diet. Clin Nutr. 2003 Feb;22(1):93-8. [PubMed:12553956 ]
    16. Geier M, Bosch OJ, Boeckh J: Ammonia as an attractive component of host odour for the yellow fever mosquito, Aedes aegypti. Chem Senses. 1999 Dec;24(6):647-53. [PubMed:10587497 ]
    17. Iwata H, Ueda Y: Pharmacokinetic considerations in development of a bioartificial liver. Clin Pharmacokinet. 2004;43(4):211-25. [PubMed:15005636 ]
    18. Ohmoto K, Miyake I, Tsuduki M, Ohno S, Yamamoto S: Control of solitary gastric fundal varices and portosystemic encephalopathy accompanying liver cirrhosis by balloon-occluded retrograde transvenous obliteration (B-RTO): a case report. Hepatogastroenterology. 1999 Mar-Apr;46(26):1249-52. [PubMed:10370701 ]
    19. Verrotti A, Greco R, Morgese G, Chiarelli F: Carnitine deficiency and hyperammonemia in children receiving valproic acid with and without other anticonvulsant drugs. Int J Clin Lab Res. 1999;29(1):36-40. [PubMed:10356662 ]
    20. Hussein HS, Flickinger EA, Fahey GC Jr: Petfood applications of inulin and oligofructose. J Nutr. 1999 Jul;129(7 Suppl):1454S-6S. [PubMed:10395620 ]
    21. Shawcross DL, Olde Damink SW, Butterworth RF, Jalan R: Ammonia and hepatic encephalopathy: the more things change, the more they remain the same. Metab Brain Dis. 2005 Sep;20(3):169-79. [PubMed:16167195 ]
    22. Albrecht J, Norenberg MD: Glutamine: a Trojan horse in ammonia neurotoxicity. Hepatology. 2006 Oct;44(4):788-94. [PubMed:17006913 ]
    23. Norenberg MD, Rama Rao KV, Jayakumar AR: Ammonia neurotoxicity and the mitochondrial permeability transition. J Bioenerg Biomembr. 2004 Aug;36(4):303-7. [PubMed:15377862 ]
    24. Brautbar N, Wu MP, Richter ED: Chronic ammonia inhalation and interstitial pulmonary fibrosis: a case report and review of the literature. Arch Environ Health. 2003 Sep;58(9):592-6. [PubMed:15369278 ]
    25. Seiler N: Ammonia and Alzheimer's disease. Neurochem Int. 2002 Aug-Sep;41(2-3):189-207. [PubMed:12020619 ]
    26. Monfort P, Kosenko E, Erceg S, Canales JJ, Felipo V: Molecular mechanism of acute ammonia toxicity: role of NMDA receptors. Neurochem Int. 2002 Aug-Sep;41(2-3):95-102. [PubMed:12020609 ]

    Only showing the first 10 proteins. There are 85 proteins in total.

    Enzymes

    General function:
    Involved in zinc ion binding
    Specific function:
    Supplies the nucleotide substrate for thymidylate synthetase.
    Gene Name:
    DCTD
    Uniprot ID:
    P32321
    Molecular weight:
    21013.96
    Reactions
    dCMP + Water → dUMP + Ammoniadetails
    General function:
    Involved in D-amino-acid oxidase activity
    Specific function:
    Selectively catalyzes the oxidative deamination of D-aspartate and its N-methylated derivative, N-methyl D-aspartate.
    Gene Name:
    DDO
    Uniprot ID:
    Q99489
    Molecular weight:
    40992.53
    Reactions
    D-Aspartic acid + Water + Oxygen → Oxalacetic acid + Ammonia + Hydrogen peroxidedetails
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Catalyzes the oxidative deamination of biogenic and xenobiotic amines and has important functions in the metabolism of neuroactive and vasoactive amines in the central nervous system and peripheral tissues. MAOB preferentially degrades benzylamine and phenylethylamine.
    Gene Name:
    MAOB
    Uniprot ID:
    P27338
    Molecular weight:
    58762.475
    Reactions
    Tryptamine + Water + Oxygen → Indoleacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Tyramine + Water + Oxygen → 4-Hydroxyphenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Aminoacetone + Water + Oxygen → Pyruvaldehyde + Ammonia + Hydrogen peroxidedetails
    Norepinephrine + Water + Oxygen → 3,4-Dihydroxymandelaldehyde + Ammonia + Hydrogen peroxidedetails
    Phenylethylamine + Oxygen + Water → Phenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Serotonin + Water + Oxygen → 5-Hydroxyindoleacetaldehyde + Ammonia + Hydrogen peroxidedetails
    N-Acetylputrescine + Water + Oxygen → N4-Acetylaminobutanal + Ammonia + Hydrogen peroxidedetails
    Dopamine + Water + Oxygen → 3,4-Dihydroxyphenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    1-Methylhistamine + Water + Oxygen → Methylimidazole acetaldehyde + Ammonia + Hydrogen peroxidedetails
    3-Methoxytyramine + Water + Oxygen → Homovanillin + Hydrogen peroxide + Ammoniadetails
    Normetanephrine + Water + Oxygen → 3-Methoxy-4-hydroxyphenylglycolaldehyde + Ammonia + Hydrogen peroxidedetails
    3-Hydroxykynurenamine + Oxygen → Quinoline-4,8-diol + Ammonia + Hydrogen peroxidedetails
    5-Hydroxykynurenamine + Water + Oxygen → 4,6-Dihydroxyquinoline + Ammonia + Hydrogen peroxide + Waterdetails
    Didemethylcitalopram + Water + Oxygen → Citalopram aldehyde + Ammonia + Hydrogen peroxidedetails
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Catalyzes the oxidative deamination of biogenic and xenobiotic amines and has important functions in the metabolism of neuroactive and vasoactive amines in the central nervous system and peripheral tissues. MAOA preferentially oxidizes biogenic amines such as 5-hydroxytryptamine (5-HT), norepinephrine and epinephrine.
    Gene Name:
    MAOA
    Uniprot ID:
    P21397
    Molecular weight:
    59681.27
    Reactions
    Tryptamine + Water + Oxygen → Indoleacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Tyramine + Water + Oxygen → 4-Hydroxyphenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Aminoacetone + Water + Oxygen → Pyruvaldehyde + Ammonia + Hydrogen peroxidedetails
    Norepinephrine + Water + Oxygen → 3,4-Dihydroxymandelaldehyde + Ammonia + Hydrogen peroxidedetails
    Phenylethylamine + Oxygen + Water → Phenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Serotonin + Water + Oxygen → 5-Hydroxyindoleacetaldehyde + Ammonia + Hydrogen peroxidedetails
    N-Acetylputrescine + Water + Oxygen → N4-Acetylaminobutanal + Ammonia + Hydrogen peroxidedetails
    Dopamine + Water + Oxygen → 3,4-Dihydroxyphenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    1-Methylhistamine + Water + Oxygen → Methylimidazole acetaldehyde + Ammonia + Hydrogen peroxidedetails
    3-Methoxytyramine + Water + Oxygen → Homovanillin + Hydrogen peroxide + Ammoniadetails
    Normetanephrine + Water + Oxygen → 3-Methoxy-4-hydroxyphenylglycolaldehyde + Ammonia + Hydrogen peroxidedetails
    3-Hydroxykynurenamine + Oxygen → Quinoline-4,8-diol + Ammonia + Hydrogen peroxidedetails
    5-Hydroxykynurenamine + Water + Oxygen → 4,6-Dihydroxyquinoline + Ammonia + Hydrogen peroxide + Waterdetails
    Didemethylcitalopram + Water + Oxygen → Citalopram aldehyde + Ammonia + Hydrogen peroxidedetails
    General function:
    Involved in D-amino-acid oxidase activity
    Specific function:
    Regulates the level of the neuromodulator D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D-amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids.
    Gene Name:
    DAO
    Uniprot ID:
    P14920
    Molecular weight:
    39473.75
    Reactions
    A D-amino acid + Water + Oxygen → a 2-oxo acid + Ammonia + Hydrogen peroxidedetails
    Glycine + Water + Oxygen → Glyoxylic acid + Ammonia + Hydrogen peroxidedetails
    D-Ornithine + Water + Oxygen → 5-Amino-2-oxopentanoic acid + Ammonia + Hydrogen peroxidedetails
    Cephalosporin C + Water + Oxygen → (7R)-7-(5-Carboxy-5-oxopentanoyl)aminocephalosporinate + Ammonia + Hydrogen peroxidedetails
    General function:
    Involved in carbohydrate metabolic process
    Specific function:
    Seems to trigger calcium oscillations in mammalian eggs. These oscillations serve as the essential trigger for egg activation and early development of the embryo (By similarity).
    Gene Name:
    GNPDA1
    Uniprot ID:
    P46926
    Molecular weight:
    32668.29
    Reactions
    Glucosamine 6-phosphate + Water → Fructose 6-phosphate + Ammoniadetails
    General function:
    Involved in hydroxymethylbilane synthase activity
    Specific function:
    Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
    Gene Name:
    HMBS
    Uniprot ID:
    P08397
    Molecular weight:
    39329.74
    Reactions
    Porphobilinogen + Water → Hydroxymethylbilane + Ammoniadetails
    General function:
    Involved in catalytic activity
    Specific function:
    Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
    Gene Name:
    CPS1
    Uniprot ID:
    P31327
    Molecular weight:
    165649.075
    Reactions
    Adenosine triphosphate + Ammonia + CO(2) + Water → ADP + Phosphoric acid + Carbamoyl phosphatedetails
    Adenosine triphosphate + Ammonia + Carbon dioxide + Water → ADP + Phosphoric acid + Carbamoyl phosphatedetails
    General function:
    Involved in copper ion binding
    Specific function:
    Catalyzes the degradation of compounds such as putrescine, histamine, spermine, and spermidine, substances involved in allergic and immune responses, cell proliferation, tissue differentiation, tumor formation, and possibly apoptosis. Placental DAO is thought to play a role in the regulation of the female reproductive function.
    Gene Name:
    ABP1
    Uniprot ID:
    P19801
    Molecular weight:
    85377.1
    Reactions
    Histamine + Water + Oxygen → Imidazole-4-acetaldehyde + Ammonia + Hydrogen peroxidedetails
    Putrescine + Oxygen + Water → 4-Aminobutyraldehyde + Ammonia + Hydrogen peroxidedetails
    Tryptamine + Water + Oxygen → Indoleacetaldehyde + Ammonia + Hydrogen peroxidedetails
    General function:
    Involved in copper ion binding
    Specific function:
    Cell adhesion protein that participates in lymphocyte recirculation by mediating the binding of lymphocytes to peripheral lymph node vascular endothelial cells in an L-selectin-independent fashion. Has a monoamine oxidase activity. May play a role in adipogenesis.
    Gene Name:
    AOC3
    Uniprot ID:
    Q16853
    Molecular weight:
    84621.27
    Reactions
    RCH(2)NH(2) + Water + Oxygen → RCHO + Ammonia + Hydrogen peroxidedetails
    Tyramine + Water + Oxygen → 4-Hydroxyphenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Aminoacetone + Water + Oxygen → Pyruvaldehyde + Ammonia + Hydrogen peroxidedetails
    Phenylethylamine + Oxygen + Water → Phenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    1,3-Diaminopropane + Oxygen + Water → 3-Aminopropionaldehyde + Ammonia + Hydrogen peroxidedetails
    N-Methylputrescine + Oxygen + Hydrogen Ion → 1-Methylpyrrolinium + Hydrogen peroxide + Ammoniadetails
    Dopamine + Water + Oxygen → 3,4-Dihydroxyphenylacetaldehyde + Ammonia + Hydrogen peroxidedetails
    Methylamine + Oxygen + Water → Formaldehyde + Ammonia + Hydrogen peroxidedetails
    Cadaverine + Water + Oxygen → 5-Aminopentanal + Ammonia + Hydrogen peroxidedetails

    Only showing the first 10 proteins. There are 85 proteins in total.