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Human Metabolome Database Version 3.5

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Showing metabocard for Ammonia (HMDB00051)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:49 -0700
HMDB ID HMDB00051
Secondary Accession Numbers None
Metabolite Identification
Common Name Ammonia
Description Ammonia is a colorless alkaline gas with a characteristic sharp smell. Ammonia is one of the most abundant nitrogen-containing compounds in the atmosphere. It is an irritant with a characteristic pungent odor, which 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. Ammonia has been shown to be a neurotoxin that 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 is formed in nearly all tissues and organs of the vertebrate organism; it is the most common endogenous neurotoxic compounds. Ammonia can affect the glutamatergic and GABAergic neuronal systems, the two prevailing neuronal systems of the cortical structures. Ammonia is well recognized to be central in the pathogenesis of hepatic encephalopathy and has been of importance to generations dating back to the early Egyptians. 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 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, this organ has only 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. The excessive formation of ammonia in the brains of Alzheimer's disease patients has 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: there is 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 Link_out, 16167195 Link_out, 15377862 Link_out, 15369278 Link_out, 12020619 Link_out).
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. Ammonia anhydrous
  2. Ammonia inhalant
  3. Ammonia solution strong [usan]
  4. Ammonia water
  5. Ammoniak
  6. Liquid ammonia
  7. Am-fol
  8. Ammonia
  9. Ammonia (conc 20% or greater)
  10. Ammonia gas
  11. Ammonia solution
  12. Ammonia solution strong (NF)
  13. Ammonia water (JP15)
  14. Ammoniac [french]
  15. Ammoniaca [italian]
  16. Ammoniacum gummi
  17. Ammoniak kconzentrierter
  18. Ammoniak [german]
  19. Ammoniakgas
  20. Ammonium ion
  21. Amoniak [polish]
  22. Anhydrous ammonia
  23. Aromatic ammonia vaporole
  24. Azane
  25. NH(3)
  26. NH3
  27. Nitro-sil
  28. Primaeres amin
  29. Sekundaeres amin
  30. Spirit of hartshorn
  31. Tertiaeres amin
Chemical Formula H3N
Average Molecular Weight 17.0305
Monoisotopic Molecular Weight 17.026549101
IUPAC Name ammonia
Traditional IUPAC Name ammonia
CAS Registry Number 7664-41-7
SMILES N
InChI Identifier InChI=1S/H3N/h1H3
InChI Key QGZKDVFQNNGYKY-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Inorganic Compounds
Super Class Homogeneous Non-metal Compounds
Class Other Non-metal Organides
Sub Class Other Non-metal Hydrides
Other Descriptors
  • a small molecule(Cyc)
  • azane(ChEBI)
  • mononuclear parent hydride(ChEBI)
Substituents
  • N/A
Direct Parent Other Non-metal Hydrides
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Alanine and aspartate metabolism
  • Component of Aminosugars metabolism
  • Component of Arginine and proline metabolism
  • Component of Cysteine metabolism
  • Component of D-Arginine and D-ornithine metabolism
  • Component of D-Glutamine and D-glutamate metabolism
  • Component of Glutamate metabolism
  • Component of Glycine, serine and threonine metabolism
  • Component of Histidine metabolism
  • Component of Methionine metabolism
  • Component of Nitrogen metabolism
  • Component of Peptidoglycan biosynthesis
  • Component of Phenylalanine metabolism
  • Component of Purine metabolism
  • Component of Pyrimidine metabolism
  • Component of Selenoamino acid metabolism
  • Component of Tryptophan metabolism
  • Component of Tyrosine metabolism
  • Component of Vitamin B6 metabolism
  • Component of beta-Alanine metabolism
Application Not Available
Cellular locations
  • Cytoplasm
Physical Properties
State Liquid
Experimental Properties
Property Value Reference
Melting Point -77.7 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 482 mg/mL at 24 °C Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
LogP -0.98 ChemAxon
pKa (strongest basic) 8.86 ChemAxon
Hydrogen Acceptor Count 1 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 13.59 A2 ChemAxon
Rotatable Bond Count 0 ChemAxon
Refractivity 15.51 ChemAxon
Polarizability 1.99 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 1 ChemAxon
Spectra
Not Available
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • All Tissues
Pathways
Name SMPDB Link KEGG Link
Glutamate Metabolism SMP00072 map00250 Link_out
Phenylalanine and Tyrosine Metabolism SMP00008 map00360 Link_out
Arginine and Proline Metabolism SMP00020 map00330 Link_out
Urea Cycle SMP00059 map00330 Link_out
Glucose-Alanine Cycle SMP00127 Not Available
Glycine and Serine Metabolism SMP00004 map00260 Link_out
D-Arginine and D-Ornithine Metabolism SMP00036 map00472 Link_out
Ammonia Recycling SMP00009 map00910 Link_out
Homocysteine Degradation SMP00455 Not Available
Amino Sugar Metabolism SMP00045 map00520 Link_out
Threonine and 2-Oxobutanoate Degradation SMP00452 Not Available
Folate Metabolism SMP00053 map00670 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
27.5 +/- 3.6 uM Newborn (0-30 days old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
29.0 (10.0-47.0) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
40.0 (0 - 80.0) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
29.0 (13.0 - 46.0) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
Article_icon
35.0 (20.0 - 58.0) uM Adult (>18 years old) Male Normal Not Available
Blood Detected and Quantified
Article_icon
29.0 (17.0 - 51.0) uM Adult (>18 years old) Female Normal Not Available
Cellular Cytoplasm Detected and Quantified
Article_icon
800 (700-900) uM Adult (>18 years old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
Article_icon
11.9 uM Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
Article_icon
2810.0 +/- 947.0 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and Quantified
Article_icon
1900.0 +/- 350.0 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Urine Detected and Quantified
2330.0 (724.0-3950.0) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
52.5 (25.0-80.0) uM Adult (>18 years old) Both Argininosuccinic aciduria (ASL) Not Available
Blood Detected and Quantified
Article_icon
1100.0 (200.0-2000.0) uM Adult (>18 years old) Both Argininosuccinic aciduria (ASL) Not Available
Blood Detected and Quantified
Article_icon
52.5 (25.0-80.0) uM Adult (>18 years old) Both Citrullinemia type I Not Available
Blood Detected and Quantified
Article_icon
1100.0 (200.0-2000.0) uM Adult (>18 years old) Both Citrullinemia Type I Not Available
Blood Detected and Quantified
Article_icon
175.0 (150.0-200.0) uM Adult (>18 years old) Both Fumaric Aciduria Not Available
Blood Detected and Quantified
Article_icon
50.4 +/- 17.0 uM Adult (>18 years old) Both Short bowel syndrome Not Available
Blood Detected and Quantified
Article_icon
52.5 (25.0-80.0) uM Adult (>18 years old) Both 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency Not Available
Blood Detected and Quantified
Article_icon
1030.0 (60.0-2000.0) uM Adult (>18 years old) Both 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency Not Available
Blood Detected and Quantified
Article_icon
52.5 (25.0-80.0) uM Adult (>18 years old) Both 3-Methyl-crotonyl-glycinuria Not Available
Blood Detected and Quantified
Article_icon
200.0 (100.0-300.0) uM Adult (>18 years old) Both 3-Methyl-Crotonyl-Glycinuria Not Available
Associated Disorders and Diseases
Disease References
Short bowel syndrome
  • 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 Link_out
    3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
      • http://www.metagene.de/program/d.prg?mp=3-HYDROXY-3-METHYLGLUTARYL-CoA%20LYASE%20DEFICIENCY
      3-methyl-crotonyl-glycinuria
        • http://www.metagene.de/program/d.prg?mp=3-METHYL-CROTONYL-GLYCINURIA
        Argininosuccinic aciduria
          • http://www.metagene.de/program/d.prg?mp=ARGININOSUCCINIC%20ACIDURIA%20(ASL)
          Citrullinemia type I
            • http://www.metagene.de/program/d.prg?id_d=92
            • http://www.metagene.de/program/d.prg?mp=CITRULLINEMIA%20TYPE%20I
            Fumarase deficiency
              • http://www.metagene.de/program/d.prg?mp=FUMARIC%20ACIDURIA
              Associated OMIM IDs
              • 246450 Link_out (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
              • 207900 Link_out (Argininosuccinic aciduria)
              • 210200 Link_out (3-methyl-crotonyl-glycinuria)
              • 215700 Link_out (Citrullinemia type I)
              • 606812 Link_out (Fumarase deficiency)
              DrugBank ID Not Available
              Phenol Explorer Compound ID Not Available
              Phenol Explorer Metabolite ID Not Available
              FoodDB ID FDB003908
              KNApSAcK ID C00007267 Link_out
              Chemspider ID 217 Link_out
              KEGG Compound ID C00014 Link_out
              BioCyc ID AMMONIA Link_out
              BiGG ID Not Available
              Wikipedia Link Ammonia Link_out
              NuGOwiki Link HMDB00051 Link_out
              Metagene Link HMDB00051 Link_out
              METLIN ID 3189 Link_out
              PubChem Compound 222 Link_out
              PDB ID NH3 Link_out
              ChEBI ID 16134 Link_out
              References
              Synthesis Reference Mohr, 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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              7. Remer T: Influence of nutrition on acid-base balance--metabolic aspects. Eur J Nutr. 2001 Oct;40(5):214-20. Pubmed: 11842946 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              17. Iwata H, Ueda Y: Pharmacokinetic considerations in development of a bioartificial liver. Clin Pharmacokinet. 2004;43(4):211-25. Pubmed: 15005636 Link_out
              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 Link_out
              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 Link_out
              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 Link_out
              Enzymes
              Name: Deoxycytidylate deaminase
              Reactions:
              • dCMP + H2O = dUMP + NH3 [RN:R01663]
              Gene Name: DCTD
              Uniprot ID: P32321 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: D-aspartate oxidase
              Reactions:
              • D-aspartate + H2O + O2 = oxaloacetate + NH3 + H2O2 [RN:R00359]
              Gene Name: DDO
              Uniprot ID: Q99489 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Amine oxidase [flavin-containing] B
              Reactions:
              • RCH2NHR' + H2O + O2 = RCHO + R'NH2 + H2O2 [RN:R01853]
              Gene Name: MAOB
              Uniprot ID: P27338 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Amine oxidase [flavin-containing] A
              Reactions:
              • RCH2NHR' + H2O + O2 = RCHO + R'NH2 + H2O2 [RN:R01853]
              Gene Name: MAOA
              Uniprot ID: P21397 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: D-amino-acid oxidase
              Reactions:
              • a D-amino acid + H2O + O2 = a 2-oxo acid + NH3 + H2O2 [RN:R01340]
              Gene Name: DAO
              Uniprot ID: P14920 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glucosamine-6-phosphate isomerase 1
              Reactions:
              • D-glucosamine 6-phosphate + H2O = D-fructose 6-phosphate + NH3 [RN:R00765]
              Gene Name: GNPDA1
              Uniprot ID: P46926 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Porphobilinogen deaminase
              Reactions:
              • 4 porphobilinogen + H2O = hydroxymethylbilane + 4 NH3 [RN:R00084]
              Gene Name: HMBS
              Uniprot ID: P08397 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Carbamoyl-phosphate synthase [ammonia], mitochondrial
              Reactions:
              • 2 ATP + NH3 + CO2 + H2O = 2 ADP + phosphate + carbamoyl phosphate [RN:R00149]
              Gene Name: CPS1
              Uniprot ID: P31327 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Amiloride-sensitive amine oxidase [copper-containing]
              Reactions:
              • histamine + H2O + O2 = (imidazol-4-yl)acetaldehyde + NH3 + H2O2 [RN:R02150]
              Gene Name: ABP1
              Uniprot ID: P19801 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Membrane primary amine oxidase
              Reactions:
              • RCH2NH2 + H2O + O2 = RCHO + NH3 + H2O2 [RN:R01853]
              Gene Name: AOC3
              Uniprot ID: Q16853 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Retina-specific copper amine oxidase
              Reactions:
              • RCH2NH2 + H2O + O2 = RCHO + NH3 + H2O2 [RN:R01853]
              Gene Name: AOC2
              Uniprot ID: O75106 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Cystathionine gamma-lyase
              Reactions:
              • (1) L-cystathionine + H2O = L-cysteine + NH3 + 2-oxobutanoate (overall reaction) [RN:R01001]
              • (2) (1a) L-cystathionine = L-cysteine + 2-ammoniobut-2-enoate [RN:R08632]
              • (3) (1b) 2-ammoniobut-2-enoate + H2O = 2-oxobutanoate + NH3 (spontaneous) [RN:R08637]
              Gene Name: CTH
              Uniprot ID: P32929 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Beta-ureidopropionase
              Reactions:
              • N-carbamoyl-beta-alanine + H2O = beta-alanine + CO2 + NH3 [RN:R00905]
              Gene Name: UPB1
              Uniprot ID: Q9UBR1 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Biotinidase
              Reactions:
              • biotin amide + H2O = biotin + NH3 [RN:R01076]
              Gene Name: BTD
              Uniprot ID: P43251 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: AMP deaminase 3
              Reactions:
              • AMP + H2O = IMP + NH3 [RN:R00181]
              Gene Name: AMPD3
              Uniprot ID: Q01432 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: AMP deaminase 2
              Reactions:
              • AMP + H2O = IMP + NH3 [RN:R00181]
              Gene Name: AMPD2
              Uniprot ID: Q01433 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: AMP deaminase 1
              Reactions:
              • AMP + H2O = IMP + NH3 [RN:R00181]
              Gene Name: AMPD1
              Uniprot ID: P23109 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Adenosine deaminase
              Reactions:
              • adenosine + H2O = inosine + NH3 [RN:R01560]
              Gene Name: ADA
              Uniprot ID: P00813 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase E
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM3
              Uniprot ID: Q08188 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutamate dehydrogenase 2, mitochondrial
              Reactions:
              • L-glutamate + H2O + NAD(P)+ = 2-oxoglutarate + NH3 + NAD(P)H + H+ [RN:R00243 R00248]
              Gene Name: GLUD2
              Uniprot ID: P49448 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase 2
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM2
              Uniprot ID: P21980 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-arginine deiminase type-6
              Reactions:
              • protein L-arginine + H2O = protein L-citrulline + NH3 [RN:R02621]
              Gene Name: PADI6
              Uniprot ID: Q6TGC4 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: L-serine dehydratase/L-threonine deaminase
              Reactions:
              • L-threonine = 2-oxobutanoate + NH3 [RN:R00996]
              Gene Name: SDS
              Uniprot ID: P20132 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Coagulation factor XIII A chain
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: F13A1
              Uniprot ID: P00488 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-arginine deiminase type-4
              Reactions:
              • protein L-arginine + H2O = protein L-citrulline + NH3 [RN:R02621]
              Gene Name: PADI4
              Uniprot ID: Q9UM07 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Pyridoxine-5'-phosphate oxidase
              Reactions:
              • (1) pyridoxamine 5'-phosphate + H2O + O2 = pyridoxal 5'-phosphate + NH3 + H2O2 [RN:R00277]
              • (2) pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2 [RN:R00278]
              Gene Name: PNPO
              Uniprot ID: Q9NVS9 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutaminase liver isoform, mitochondrial
              Reactions:
              • L-glutamine + H2O = L-glutamate + NH3 [RN:R00256]
              Gene Name: GLS2
              Uniprot ID: Q9UI32 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: CTP synthase 1
              Reactions:
              • ATP + UTP + NH3 = ADP + phosphate + CTP [RN:R00571]
              Gene Name: CTPS
              Uniprot ID: P17812 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-arginine deiminase type-3
              Reactions:
              • protein L-arginine + H2O = protein L-citrulline + NH3 [RN:R02621]
              Gene Name: PADI3
              Uniprot ID: Q9ULW8 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: GMP reductase 2
              Reactions:
              • inosine 5'-phosphate + NH3 + NADP+ = guanosine 5'-phosphate + NADPH + H+ [RN:R01134]
              Gene Name: GMPR2
              Uniprot ID: Q9P2T1 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase 5
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM5
              Uniprot ID: O43548 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutaminase kidney isoform, mitochondrial
              Reactions:
              • L-glutamine + H2O = L-glutamate + NH3 [RN:R00256]
              Gene Name: GLS
              Uniprot ID: O94925 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase 4
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM4
              Uniprot ID: P49221 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase K
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM1
              Uniprot ID: P22735 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Histidine ammonia-lyase
              Reactions:
              • L-histidine = urocanate + NH3 [RN:R01168]
              Gene Name: HAL
              Uniprot ID: P42357 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-arginine deiminase type-2
              Reactions:
              • protein L-arginine + H2O = protein L-citrulline + NH3 [RN:R02621]
              Gene Name: PADI2
              Uniprot ID: Q9Y2J8 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: GMP reductase 1
              Reactions:
              • inosine 5'-phosphate + NH3 + NADP+ = guanosine 5'-phosphate + NADPH + H+ [RN:R01134]
              Gene Name: GMPR
              Uniprot ID: P36959 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Formimidoyltransferase-cyclodeaminase
              Reactions:
              • 5-formimidoyltetrahydrofolate = 5,10-methenyltetrahydrofolate + NH3 [RN:R02302]
              Gene Name: FTCD
              Uniprot ID: O95954 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-arginine deiminase type-1
              Reactions:
              • protein L-arginine + H2O = protein L-citrulline + NH3 [RN:R02621]
              Gene Name: PADI1
              Uniprot ID: Q9ULC6 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aminomethyltransferase, mitochondrial
              Reactions:
              • [protein]-S8-aminomethyldihydrolipoyllysine + tetrahydrofolate = [protein]-dihydrolipoyllysine + 5,10-methylenetetrahydrofolate + NH3 [RN:R04125]
              Gene Name: AMT
              Uniprot ID: P48728 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Guanine deaminase
              Reactions:
              • guanine + H2O = xanthine + NH3 [RN:R01676]
              Gene Name: GDA
              Uniprot ID: Q9Y2T3 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase 6
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM6
              Uniprot ID: O95932 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutaminyl-peptide cyclotransferase
              Reactions:
              • L-glutaminyl-peptide = 5-oxoprolyl-peptide + NH3 [RN:R04058]
              Gene Name: QPCT
              Uniprot ID: Q16769 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-glutamine gamma-glutamyltransferase Z
              Reactions:
              • protein glutamine + alkylamine = protein N5-alkylglutamine + NH3 [RN:R03983]
              Gene Name: TGM7
              Uniprot ID: Q96PF1 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutamate dehydrogenase 1, mitochondrial
              Reactions:
              • L-glutamate + H2O + NAD(P)+ = 2-oxoglutarate + NH3 + NAD(P)H + H+ [RN:R00243 R00248]
              Gene Name: GLUD1
              Uniprot ID: P00367 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Protein-lysine 6-oxidase
              Reactions:
              • peptidyl-L-lysyl-peptide + O2 + H2O = peptidyl-allysyl-peptide + NH3 + H2O2 [RN:R04239]
              Gene Name: LOX
              Uniprot ID: P28300 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Ammonium transporter Rh type A
              Reactions:
                Gene Name: RHAG
                Uniprot ID: Q02094 Link_out
                Protein Sequence: FASTA
                Gene Sequence: FASTA
                Name: AID
                Reactions:
                  Gene Name: AID
                  Uniprot ID: Q546Y9 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: CAD protein
                  Reactions:
                  • (1) 2 ATP + L-glutamine + HCO3- + H2O = 2 ADP + phosphate + L-glutamate + carbamoyl phosphate [RN:R00575]
                  • (2) L-glutamine + H2O = L-glutamate + NH3 [RN:R00256]
                  • (3) 2 ATP + HCO3- = 2 ADP + phosphate + carbamoyl phosphate [RN:R07641]
                  Gene Name: CAD
                  Uniprot ID: P27708 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: L-asparaginase
                  Reactions:
                  • L-asparagine + H2O = L-aspartate + NH3 [RN:R00485]
                  Gene Name: ASRGL1
                  Uniprot ID: Q7L266 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: CTP synthase 2
                  Reactions:
                  • ATP + UTP + NH3 = ADP + phosphate + CTP [RN:R00571]
                  Gene Name: CTPS2
                  Uniprot ID: Q9NRF8 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: Glutamine synthetase
                  Reactions:
                  • ATP + L-glutamate + NH3 = ADP + phosphate + L-glutamine [RN:R00253]
                  Gene Name: PIG59
                  Uniprot ID: A8YXX4 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: Glucosamine-6-phosphate isomerase 2
                  Reactions:
                  • D-glucosamine 6-phosphate + H2O = D-fructose 6-phosphate + NH3 [RN:R00765]
                  Gene Name: GNPDA2
                  Uniprot ID: Q8TDQ7 Link_out
                  Protein Sequence: FASTA
                  Gene Sequence: FASTA
                  Name: Ammonium transporter Rh type C
                  Reactions:
                    Gene Name: RHCG
                    Uniprot ID: Q9UBD6 Link_out
                    Protein Sequence: FASTA
                    Gene Sequence: FASTA
                    Name: Ammonium transporter Rh type B
                    Reactions:
                      Gene Name: RHBG
                      Uniprot ID: Q9H310 Link_out
                      Protein Sequence: FASTA
                      Gene Sequence: FASTA