| 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 , 16167195 , 15377862 , 15369278 , 12020619 ). |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
|
| Synonyms |
- Ammonia anhydrous
- Ammonia inhalant
- Ammonia solution strong [usan]
- Ammonia water
- Ammoniak
- Liquid ammonia
- Am-fol
- Ammonia
- Ammonia (conc 20% or greater)
- Ammonia gas
- Ammonia solution
- Ammonia solution strong (NF)
- Ammonia water (JP15)
- Ammoniac [french]
- Ammoniaca [italian]
- Ammoniacum gummi
- Ammoniak kconzentrierter
- Ammoniak [german]
- Ammoniakgas
- Ammonium ion
- Amoniak [polish]
- Anhydrous ammonia
- Aromatic ammonia vaporole
- Azane
- NH(3)
- NH3
- Nitro-sil
- Primaeres amin
- Sekundaeres amin
- Spirit of hartshorn
- 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 |
|
| Direct Parent |
Other Non-metal Hydrides |
| Ontology |
| Status |
Detected and Quantified |
| Origin |
|
| 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 |
|
| 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 |
|
| Spectra |
|
Not Available
|
| Biological Properties |
| Cellular Locations |
|
| Biofluid Locations |
- Blood
- Cellular Cytoplasm
- Cerebrospinal Fluid (CSF)
- Urine
|
| Tissue Location |
|
| Pathways |
|
| Normal Concentrations |
|
| Blood |
Detected and Quantified |
|
27.5 +/- 3.6 uM |
Newborn (0-30 days old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
29.0 (10.0-47.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
40.0 (0 - 80.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
29.0 (13.0 - 46.0) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
35.0 (20.0 - 58.0) uM |
Adult (>18 years old) |
Male |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
29.0 (17.0 - 51.0) uM |
Adult (>18 years old) |
Female |
Normal |
Not Available |
| Cellular Cytoplasm |
Detected and Quantified |
|
800 (700-900) uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
11.9 uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
2810.0 +/- 947.0 umol/mmol creatinine |
Adult (>18 years old) |
Male |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
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 |
|
| Blood |
Detected and Quantified |
|
52.5 (25.0-80.0) uM |
Adult (>18 years old) |
Both |
Argininosuccinic aciduria (ASL) |
Not Available |
| Blood |
Detected and Quantified |
|
1100.0 (200.0-2000.0) uM |
Adult (>18 years old) |
Both |
Argininosuccinic aciduria (ASL) |
Not Available |
| Blood |
Detected and Quantified |
|
52.5 (25.0-80.0) uM |
Adult (>18 years old) |
Both |
Citrullinemia type I |
Not Available |
| Blood |
Detected and Quantified |
|
1100.0 (200.0-2000.0) uM |
Adult (>18 years old) |
Both |
Citrullinemia Type I |
Not Available |
| Blood |
Detected and Quantified |
|
175.0 (150.0-200.0) uM |
Adult (>18 years old) |
Both |
Fumaric Aciduria |
Not Available |
| Blood |
Detected and Quantified |
|
50.4 +/- 17.0 uM |
Adult (>18 years old) |
Both |
Short bowel syndrome |
Not Available |
| Blood |
Detected and Quantified |
|
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 |
|
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 |
|
52.5 (25.0-80.0) uM |
Adult (>18 years old) |
Both |
3-Methyl-crotonyl-glycinuria |
Not Available |
| Blood |
Detected and Quantified |
|
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
|
| 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
(3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
- 207900
(Argininosuccinic aciduria)
- 210200
(3-methyl-crotonyl-glycinuria)
- 215700
(Citrullinemia type I)
- 606812
(Fumarase deficiency)
|
| External Links |
| DrugBank ID |
Not Available |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB003908 |
| KNApSAcK ID |
C00007267  |
| Chemspider ID |
217  |
| KEGG Compound ID |
C00014  |
| BioCyc ID |
AMMONIA  |
| BiGG ID |
Not Available |
| Wikipedia Link |
Ammonia  |
| NuGOwiki Link |
HMDB00051  |
| Metagene Link |
HMDB00051  |
| METLIN ID |
3189  |
| PubChem Compound |
222  |
| PDB ID |
NH3  |
| ChEBI ID |
16134  |
| 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 |
- 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
- 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
- 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
- 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
- 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
- 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
- Remer T: Influence of nutrition on acid-base balance--metabolic aspects. Eur J Nutr. 2001 Oct;40(5):214-20.
Pubmed: 11842946
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- Iwata H, Ueda Y: Pharmacokinetic considerations in development of a bioartificial liver. Clin Pharmacokinet. 2004;43(4):211-25.
Pubmed: 15005636
- 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
- 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
- Hussein HS, Flickinger EA, Fahey GC Jr: Petfood applications of inulin and oligofructose. J Nutr. 1999 Jul;129(7 Suppl):1454S-6S.
Pubmed: 10395620
|
| Enzymes |
|
|
| Name: |
D-aspartate oxidase
|
| Reactions: |
- D-aspartate + H2O + O2 = oxaloacetate + NH3 + H2O2 [RN:R00359]
|
| Gene Name: |
DDO |
| Uniprot ID: |
Q99489  |
| 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  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
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| 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  |
| 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  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
Biotinidase
|
| Reactions: |
- biotin amide + H2O = biotin + NH3 [RN:R01076]
|
| Gene Name: |
BTD |
| Uniprot ID: |
P43251  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
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|
|
| 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  |
| 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  |
| 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  |
| 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  |
| 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  |
| 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  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
|
|
| Name: |
AID
|
| Reactions: |
|
| Gene Name: |
AID |
| Uniprot ID: |
Q546Y9  |
| 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  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
| Name: |
L-asparaginase
|
| Reactions: |
- L-asparagine + H2O = L-aspartate + NH3 [RN:R00485]
|
| Gene Name: |
ASRGL1 |
| Uniprot ID: |
Q7L266  |
| 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  |
| 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  |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
|
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