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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 08:53:34 UTC
HMDB IDHMDB0000595
Secondary Accession Numbers
  • HMDB00595
Metabolite Identification
Common NameHydrogen carbonate
DescriptionBicarbonate, or hydrogen carbonate, is a simple single carbon molecule that plays surprisingly important roles in diverse biological processes. Among these are photosynthesis, the Krebs cycle, whole-body and cellular pH regulation, and volume regulation. Since bicarbonate is charged it is not permeable to lipid bilayers. Mammalian membranes thus contain bicarbonate transport proteins to facilitate the specific transmembrane movement of HCO3(-). Bicarbonate ion is an anion that consists of one central carbon atom surrounded by three oxygen atoms in a trigonal planar arrangement, with a hydrogen atom attached to one of the oxygens. The bicarbonate ion carries a negative one formal charge and is the conjugate base of carbonic acid, H2CO3. The carbonate radical is an elusive and strong one-electron oxidant. Bicarbonate in equilibrium with carbon dioxide constitutes the main physiological buffer. The bicarbonate-carbon dioxide pair stimulates the oxidation, peroxidation and nitration of several biological targets. The demonstration that the carbonate radical existed as an independent species in aqueous solutions at physiological pH and temperature renewed the interest in the pathophysiological roles of this radical and related species. The carbonate radical has been proposed to be a key mediator of the oxidative damage resulting from peroxynitrite production, xanthine oxidase turnover and superoxide dismutase1 peroxidase activity. The carbonate radical has also been proposed to be responsible for the stimulatory effects of the bicarbonate-carbon dioxide pair on oxidations mediated by hydrogen peroxide/transition metal ions. The ultimate precursor of the carbonate radical anion being bicarbonate, carbon dioxide, peroxymonocarbonate or complexes of transition metal ions with bicarbonate-derived species remains a matter of debate. The carbonate radical mediates some of the pathogenic effects of peroxynitrite. The carbonate radical as the oxidant produced from superoxide dismutase (EC 1.15.1.1, SOD1) peroxidase activity. Peroxymonocarbonate is a biological oxidant, whose existence is in equilibrium with hydrogen peroxide and bicarbonate (PMID: 17505962 , 17215880 ). Hydrogen carbonate is found to be associated with hawkinsinuria, which is an inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
[co(OH)2]ChEBI
Dihydrogen carbonateChEBI
H2CO3ChEBI
KoehlensaeureChEBI
CarbonateGenerator
Dihydrogen carbonic acidGenerator
Acid OF airHMDB
Aerial acidHMDB
Bisodium carbonateHMDB
CalcinedHMDB
Carbonic acid sodium saltHMDB
ConsalHMDB
Crystol carbonateHMDB
Disodium carbonateHMDB
Mild alkaliHMDB
Na-XHMDB
OxyperHMDB
Sal sodaHMDB
Salt OF sodaHMDB
Scotch sodaHMDB
SodaHMDB
Soda ashHMDB
Sodium carbonateHMDB
Sodium carbonate anhydrousHMDB
Sodium carbonate hydratedHMDB
Sodium carbonate peroxyhydrateHMDB
Solvay sodaHMDB
Trona soda ashHMDB
Tronalight light soda ashHMDB
Acid, carbonicMeSH
Chemical FormulaCH2O3
Average Molecular Weight62.0248
Monoisotopic Molecular Weight62.00039393
IUPAC Namecarbonic acid
Traditional Namecarbonic acid
CAS Registry Number71-52-3
SMILES
OC(O)=O
InChI Identifier
InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4)
InChI KeyBVKZGUZCCUSVTD-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as organic carbonic acids. These are compounds comprising the carbonic acid functional group.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassOrganic carbonic acids and derivatives
Sub ClassOrganic carbonic acids
Direct ParentOrganic carbonic acids
Alternative Parents
Substituents
  • Carbonic acid
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point720 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility12.8 mg/mL (cold water)MERCK INDEX (1996)
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility732 g/LALOGPS
logP0.6ALOGPS
logP0.25ChemAxon
logS0.57ALOGPS
pKa (Strongest Acidic)6.05ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 ŲChemAxon
Rotatable Bond Count0ChemAxon
Refractivity9.5 m³·mol⁻¹ChemAxon
Polarizability4.23 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, Positivesplash10-03di-9000000000-310dbbc64fba7d9c667eView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00du-9300000000-b9ab1da5629a3dfff55fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03di-9000000000-53429210d3161a8e792fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03di-9000000000-65bbb10c2768f3746b62View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03di-9000000000-b6afca3e3ac002546879View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-9000000000-0ef3797aeb5276c64c90View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-03di-9000000000-5b50453541e6f14e35beView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-03di-9000000000-5b50453541e6f14e35beView in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue LocationNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified24900.0 +/- 1790.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified24700.0 +/- 1200.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified23100.0 +/- 1500.0 uMAdult (>18 years old)Female
Normal
    • Geigy Scientific ...
details
BloodDetected and Quantified20000.0 (15500.0-22500.0) uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified20000-30000 uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified22000-26000 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified23000-26000 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified23000-26000 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified2950.00721-4425.0108 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified24000-32000 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified22000-29000 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified22000-29000 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified21000-29000 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified>20000 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified24000 (22000-26000) uMChildren (1 - 13 years old)BothNormal details
Cellular CytoplasmDetected and Quantified11200 +/- 150 uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified10000 +/- 1000 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified7600 +/- 1600 uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified11000 uMNewborn (0-30 days old)Female
21-hydroxylase deficiency
details
BloodDetected and Quantified13600.00 uMAdult (>18 years old)BothDiabetic ketoacidosis details
BloodDetected and Quantified16200 uMInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency details
BloodDetected and Quantified21000 uMInfant (0-1 year old)MaleLeigh Syndrome details
BloodDetected and Quantified3500 uMInfant (0-1 year old)MalePyruvate dehydrogenase phosphatase deficiency details
BloodDetected and Quantified17000-18000 uMChildren (1-13 years old)FemaleFanconi Bickel syndrome
    • Late Diagnosis of...
details
BloodDetected and Quantified20100 uMAdolescent (13-18 years old)Both
Renal tubular acidosis, distal, RTA type 1
details
BloodDetected and Quantified17800-19500 uMAdult (>18 years old)Both
Renal tubular acidosis, distal, RTA type 1
details
BloodDetected and Quantified4800-15000 uMInfant (0-1 year old)BothRenal tubular acidosis, distal, RTA type 1 details
BloodDetected and Quantified5500-13000 uMNewborn (0-30 days old)Both
Renal tubular acidosis, distal, RTA type 1
details
BloodDetected and Quantified6300-19500 uMChildren (1-13 years old)BothRenal tubular acidosis, distal, RTA type 1 details
BloodDetected and Quantified16900 uMNewborn (0-30 days old)Male3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency details
BloodDetected and Quantified30000-43000 uMInfant (0-1 year old)BothCongenital chloride diarrhea details
BloodDetected and Quantified28000-35000 uMAdult (>18 years old)BothGitelman syndrome details
BloodDetected and Quantified28000-33000 uMAdult (>18 years old)FemaleSeizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES) details
BloodDetected and Quantified12800 uMAdult (>18 years old)FemaleFanconi syndrome details
BloodDetected and Quantified5000-15000 uMChildren (1-13 years old)MaleMonocarboxylate transporter 1 deficiency details
BloodDetected and Quantified7000-13000 uMInfant (0-1 year old)MaleCarnitine palmitoyltransferase deficiency I details
BloodDetected and Quantified22400 +/- 700 uMChildren (1-13 years old)MaleFanconi syndrome details
BloodDetected and Quantified12700 uMAdolescent (13-18 years old)FemaleRenal tubular acidosis, proximal, with ocular abnormalities and mental retardation details
BloodDetected and Quantified9300 uMNewborn (0-30 days old)MaleSuccinyl CoA: 3-ketoacid CoA transferase deficiency details
BloodDetected and Quantified9700-16600 uMInfant (0-1 year old)BothCongenital secretory diarrhea details
BloodDetected and Quantified2130.561 uMNewborn (0-30 days old)Not AvailablePseudohypoaldosteronism, type I, autosomal dominant details
BloodDetected and Quantified15000 uMNewborn (0-30 days old)Male2-Ketoglutarate dehydrogenase complex deficiency details
BloodDetected and Quantified11000-21000 uMChildren (1-13 years old)Both2-Ketoglutarate dehydrogenase complex deficiency details
BloodDetected and Quantified18000 uMNewborn (0-30 days old)FemaleBartter Syndrome, Type 2, Antenatal details
BloodDetected and Quantified25500.00 (24000.00-27000.00) uMAdult (>18 years old)BothHawkinsinuria
    • MetaGene: Metabol...
details
BloodDetected and Quantified12000.00 (10000.00-14000.00) uMChildren (1-13 years old)BothHawkinsinuria
    • MetaGene: Metabol...
details
BloodDetected and Quantified19800-28300 uMNewborn (0-30 days old)FemaleBartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness details
BloodDetected and Quantified23900 uMInfant (0-1 year old)FemaleBartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness details
BloodDetected and Quantified25900-37200 uMChildren (1-13 years old)FemaleBartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness details
BloodDetected and Quantified33500 uMInfant (0-1 year old)Not Specified
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
details
BloodDetected and Quantified24000-38000 uMNewborn (0-30 days old)MaleBartter Syndrome, Type 5, Antenatal, Transient details
BloodDetected and Quantified38000 uMNewborn (0-30 days old)Female
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
details
BloodDetected and Quantified18900 +/- 2500 uMAdolescent (13-18 years old)BothLeigh Syndrome, French Canadian Type details
BloodDetected and Quantified16000 uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
BloodDetected and Quantified11000-15000 uMNewborn (0-30 days old)MaleNarp Syndrome details
BloodDetected and Quantified23000 uMAdult (>18 years old)Male
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
details
UrineDetected and Quantified0 umol/mmol creatinineChildren (1-13 years old)MaleFanconi syndrome details
UrineDetected and Quantified3060 umol/mmol creatinineAdolescent (13-18 years old)FemaleRenal tubular acidosis, proximal, with ocular abnormalities and mental retardation details
Associated Disorders and Diseases
Disease References
21-Hydroxylase deficiency
  1. Warinner SA, Zimmerman D, Thompson GB, Grant CS: Study of three patients with congenital adrenal hyperplasia treated by bilateral adrenalectomy. World J Surg. 2000 Nov;24(11):1347-52. [PubMed:11038205 ]
2-Ketoglutarate dehydrogenase complex deficiency
  1. Guffon N, Lopez-Mediavilla C, Dumoulin R, Mousson B, Godinot C, Carrier H, Collombet JM, Divry P, Mathieu M, Guibaud P: 2-Ketoglutarate dehydrogenase deficiency, a rare cause of primary hyperlactataemia: report of a new case. J Inherit Metab Dis. 1993;16(5):821-30. [PubMed:8295396 ]
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. 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 ]
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 ]
Hawkinsinuria
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Ketoacidosis
  1. Snyderman SE, Sansaricq C, Middleton B: Succinyl-CoA:3-ketoacid CoA-transferase deficiency. Pediatrics. 1998 Apr;101(4 Pt 1):709-11. [PubMed:9521962 ]
Fanconi syndrome
  1. Cheng HM, Jap TS, Ho LT: Fanconi syndrome: report of a case. J Formos Med Assoc. 1990 Dec;89(12):1115-7. [PubMed:1982686 ]
  2. McSherry E, Sebastian A, Morris RC Jr: Renal tubular acidosis in infants: the several kinds, including bicarbonate-wasting, classic renal tubular acidosis. J Clin Invest. 1972 Mar;51(3):499-514. [PubMed:5011097 ]
Renal tubular acidosis, distal, RTA type 1
  1. Karet FE, Gainza FJ, Gyory AZ, Unwin RJ, Wrong O, Tanner MJ, Nayir A, Alpay H, Santos F, Hulton SA, Bakkaloglu A, Ozen S, Cunningham MJ, di Pietro A, Walker WG, Lifton RP: Mutations in the chloride-bicarbonate exchanger gene AE1 cause autosomal dominant but not autosomal recessive distal renal tubular acidosis. Proc Natl Acad Sci U S A. 1998 May 26;95(11):6337-42. [PubMed:9600966 ]
Leigh's syndrome, subacute necrotizing encephalopathy, SNE
  1. Hommes FA, Polman HA, Reerink JD: Leigh's encephalomyelopathy: an inborn error of gluconeogenesis. Arch Dis Child. 1968 Aug;43(230):423-6. [PubMed:4873809 ]
Bartter Syndrome, Type 2, Antenatal
  1. Chan WK, To KF, Tong JH, Law CW: Paradoxical hypertension and salt wasting in Type II Bartter syndrome. Clin Kidney J. 2012 Jun;5(3):217-20. doi: 10.1093/ckj/sfs026. Epub 2012 Mar 29. [PubMed:26069767 ]
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
  1. Zaffanello M, Taranta A, Palma A, Bettinelli A, Marseglia GL, Emma F: Type IV Bartter syndrome: report of two new cases. Pediatr Nephrol. 2006 Jun;21(6):766-70. doi: 10.1007/s00467-006-0090-x. Epub 2006 Apr 1. [PubMed:16583241 ]
  2. Heilberg IP, Totoli C, Calado JT: Adult presentation of Bartter syndrome type IV with erythrocytosis. Einstein (Sao Paulo). 2015 Oct-Dec;13(4):604-6. doi: 10.1590/S1679-45082015RC3013. Epub 2015 Oct 30. [PubMed:26537508 ]
Fanconi Bickel syndrome
  1. Nirupama Gupta, Bimota Nambam, David A. Weinstein, and Lawrence R. Shoemaker (2016). Late Diagnosis of Fanconi-Bickel Syndrome: Challenges With the Diagnosis and Literature Review . Journal of Inborn Errors of Metabolism and Screening.
Bartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness
  1. Nozu K, Inagaki T, Fu XJ, Nozu Y, Kaito H, Kanda K, Sekine T, Igarashi T, Nakanishi K, Yoshikawa N, Iijima K, Matsuo M: Molecular analysis of digenic inheritance in Bartter syndrome with sensorineural deafness. J Med Genet. 2008 Mar;45(3):182-6. doi: 10.1136/jmg.2007.052944. [PubMed:18310267 ]
Bartter Syndrome, Type 5, Antenatal, Transient
  1. Laghmani K, Beck BB, Yang SS, Seaayfan E, Wenzel A, Reusch B, Vitzthum H, Priem D, Demaretz S, Bergmann K, Duin LK, Gobel H, Mache C, Thiele H, Bartram MP, Dombret C, Altmuller J, Nurnberg P, Benzing T, Levtchenko E, Seyberth HW, Klaus G, Yigit G, Lin SH, Timmer A, de Koning TJ, Scherjon SA, Schlingmann KP, Bertrand MJ, Rinschen MM, de Backer O, Konrad M, Komhoff M: Polyhydramnios, Transient Antenatal Bartter's Syndrome, and MAGED2 Mutations. N Engl J Med. 2016 May 12;374(19):1853-63. doi: 10.1056/NEJMoa1507629. Epub 2016 Apr 27. [PubMed:27120771 ]
Congenital chloride diarrhea
  1. Choi M, Scholl UI, Ji W, Liu T, Tikhonova IR, Zumbo P, Nayir A, Bakkaloglu A, Ozen S, Sanjad S, Nelson-Williams C, Farhi A, Mane S, Lifton RP: Genetic diagnosis by whole exome capture and massively parallel DNA sequencing. Proc Natl Acad Sci U S A. 2009 Nov 10;106(45):19096-101. doi: 10.1073/pnas.0910672106. Epub 2009 Oct 27. [PubMed:19861545 ]
Congenital secretory diarrhea
  1. Muller T, Wijmenga C, Phillips AD, Janecke A, Houwen RH, Fischer H, Ellemunter H, Fruhwirth M, Offner F, Hofer S, Muller W, Booth IW, Heinz-Erian P: Congenital sodium diarrhea is an autosomal recessive disorder of sodium/proton exchange but unrelated to known candidate genes. Gastroenterology. 2000 Dec;119(6):1506-13. [PubMed:11113072 ]
Dihydrolipoamide Dehydrogenase Deficiency
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Gitelman syndrome
  1. Lin SH, Cheng NL, Hsu YJ, Halperin ML: Intrafamilial phenotype variability in patients with Gitelman syndrome having the same mutations in their thiazide-sensitive sodium/chloride cotransporter. Am J Kidney Dis. 2004 Feb;43(2):304-12. [PubMed:14750096 ]
Leigh Syndrome, French Canadian Type
  1. Debray FG, Morin C, Janvier A, Villeneuve J, Maranda B, Laframboise R, Lacroix J, Decarie JC, Robitaille Y, Lambert M, Robinson BH, Mitchell GA: LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiency. J Med Genet. 2011 Mar;48(3):183-9. doi: 10.1136/jmg.2010.081976. Epub 2011 Jan 25. [PubMed:21266382 ]
Lipoyltransferase 1 Deficiency
  1. Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Monocarboxylate transporter 1 deficiency
  1. Balasubramaniam S, Lewis B, Greed L, Meili D, Flier A, Yamamoto R, Bilic K, Till C, Sass JO: Heterozygous Monocarboxylate Transporter 1 (MCT1, SLC16A1) Deficiency as a Cause of Recurrent Ketoacidosis. JIMD Rep. 2016;29:33-38. doi: 10.1007/8904_2015_519. Epub 2015 Nov 26. [PubMed:26608392 ]
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 ]
Pseudohypoaldosteronism, type I, autosomal dominant
  1. Bowden SA, Cozzi C, Hickey SE, Thrush DL, Astbury C, Nuthakki S: Autosomal dominant pseudohypoaldosteronism type 1 in an infant with salt wasting crisis associated with urinary tract infection and obstructive uropathy. Case Rep Endocrinol. 2013;2013:524647. doi: 10.1155/2013/524647. Epub 2013 Dec 19. [PubMed:24455331 ]
Pyruvate dehydrogenase phosphatase deficiency
  1. Robinson BH, Sherwood WG: Pyruvate dehydrogenase phosphatase deficiency: a cause of congenital chronic lactic acidosis in infancy. Pediatr Res. 1975 Dec;9(12):935-9. doi: 10.1203/00006450-197512000-00015. [PubMed:172850 ]
Renal tubular acidosis, proximal, with ocular abnormalities and mental retardation
  1. Igarashi T, Ishii T, Watanabe K, Hayakawa H, Horio K, Sone Y, Ohga K: Persistent isolated proximal renal tubular acidosis--a systemic disease with a distinct clinical entity. Pediatr Nephrol. 1994 Feb;8(1):70-1. [PubMed:8142230 ]
Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES)
  1. Scholl UI, Choi M, Liu T, Ramaekers VT, Hausler MG, Grimmer J, Tobe SW, Farhi A, Nelson-Williams C, Lifton RP: Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SeSAME syndrome) caused by mutations in KCNJ10. Proc Natl Acad Sci U S A. 2009 Apr 7;106(14):5842-7. doi: 10.1073/pnas.0901749106. Epub 2009 Mar 16. [PubMed:19289823 ]
Associated OMIM IDs
  • 140350 (Hawkinsinuria)
  • 179800 (Renal tubular acidosis, distal, RTA type 1)
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 201910 (21-Hydroxylase deficiency)
  • 602522 (Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 241200 (Bartter Syndrome, Type 2, Antenatal)
  • 246900 (Dihydrolipoamide Dehydrogenase Deficiency)
  • 256000 (Leigh's syndrome, subacute necrotizing encephalopathy, SNE)
  • 608782 (Pyruvate dehydrogenase phosphatase deficiency)
  • 177735 (Pseudohypoaldosteronism, type I, autosomal dominant)
  • 214700 (Congenital chloride diarrhea)
  • 263800 (Gitelman syndrome)
  • 612780 (Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES))
  • 616095 (Monocarboxylate transporter 1 deficiency)
  • 255120 (Carnitine palmitoyltransferase I deficiency)
  • 604278 (Renal tubular acidosis, proximal, with ocular abnormalities and mental retardation)
  • 245050 (Ketoacidosis)
  • 270420 (Congenital secretory diarrhea)
  • 613090 (Bartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness)
  • 300971 (Bartter Syndrome, Type 5, Antenatal, Transient)
  • 220111 (Leigh Syndrome, French Canadian Type)
  • 616299 (Lipoyltransferase 1 Deficiency)
  • 600462 (Myopathy, lactic acidosis, and sideroblastic anemia 1)
  • 227810 (Fanconi Bickel syndrome)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB023191
KNApSAcK IDNot Available
Chemspider ID747
KEGG Compound IDC01353
BioCyc IDCARBON-DIOXIDE
BiGG ID1436647
Wikipedia LinkCarbonic acid
METLIN ID6944
PubChem Compound767
PDB IDNot Available
ChEBI ID28976
References
Synthesis ReferenceNakajima, Fumiaki; Arima, Toshikazu; Kikuchi, Shintaro; Hirano, Hachiro. Production of alkaline hydrogen carbonate. Jpn. Kokai Tokkyo Koho (2005), 15 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Medinas DB, Cerchiaro G, Trindade DF, Augusto O: The carbonate radical and related oxidants derived from bicarbonate buffer. IUBMB Life. 2007 Apr-May;59(4-5):255-62. [PubMed:17505962 ]
  2. Casey JR: Why bicarbonate? Biochem Cell Biol. 2006 Dec;84(6):930-9. [PubMed:17215880 ]

Only showing the first 10 proteins. There are 21 proteins in total.

Enzymes

General function:
Lipid transport and metabolism
Specific function:
Catalyzes fat and vitamin absorption. Acts in concert with pancreatic lipase and colipase for the complete digestion of dietary triglycerides.
Gene Name:
CEL
Uniprot ID:
P19835
Molecular weight:
79666.385
General function:
Involved in inositol or phosphatidylinositol phosphatase activity
Specific function:
Converts phosphatidylinositol 4,5-bisphosphate to phosphatidylinositol 4-phosphate. Also converts inositol 1,4,5-trisphosphate to inositol 1,4-bisphosphate and inositol 1,3,4,5-tetrakisphosphate to inositol 1,3,4-trisphosphate. May function in lysosomal membrane trafficking by regulating the specific pool of phosphatidylinositol 4,5-bisphosphate that is associated with lysosomes. Involved in primary cilia assembly.
Gene Name:
OCRL
Uniprot ID:
Q01968
Molecular weight:
104203.78
General function:
Involved in ferric iron binding
Specific function:
The lactotransferrin transferrin-like domain 1 functions as a serine protease of the peptidase S60 family that cuts arginine rich regions. This function contributes to the antimicrobial activity
Gene Name:
LTF
Uniprot ID:
P02788
Molecular weight:
78181.2
General function:
Involved in ferric iron binding
Specific function:
Transferrins are iron binding transport proteins which can bind two Fe(3+) ions in association with the binding of an anion, usually bicarbonate. It is responsible for the transport of iron from sites of absorption and heme degradation to those of storage and utilization. Serum transferrin may also have a further role in stimulating cell proliferation
Gene Name:
TF
Uniprot ID:
P02787
Molecular weight:
77049.2
General function:
Involved in ATP binding
Specific function:
Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter. Can inhibit the chloride channel activity of ANO1.
Gene Name:
CFTR
Uniprot ID:
P13569
Molecular weight:
168139.895
General function:
Involved in carbonate dehydratase activity
Specific function:
Reversible hydration of carbon dioxide. May stimulate the sodium/bicarbonate transporter activity of SLC4A4 that acts in pH homeostasis. It is essential for acid overload removal from the retina and retina epithelium, and acid release in the choriocapillaris in the choroid.
Gene Name:
CA4
Uniprot ID:
P22748
Molecular weight:
35032.075
General function:
Involved in secondary active sulfate transmembrane transporter activity
Specific function:
Chloride/bicarbonate exchanger. Involved in absorbtion of in the colon. Helps mediate electrolyte and fluid absorption
Gene Name:
SLC26A3
Uniprot ID:
P40879
Molecular weight:
84504.0
General function:
Involved in phosphorus-oxygen lyase activity
Specific function:
Soluble adenylyl cyclase that has a critical role in mammalian spermatogenesis. Produces the cAMP which mediates in part the cAMP-responsive nuclear factors indispensable for maturation of sperm in the epididymis. Induces capacitation, the maturational process that sperm undergo prior to fertilization. May be the bicarbonate sensor. Involved in ciliary beat regulation.
Gene Name:
ADCY10
Uniprot ID:
Q96PN6
Molecular weight:
169748.45
General function:
Involved in secondary active sulfate transmembrane transporter activity
Specific function:
DIDS- and thiosulfate- sensitive anion exchanger mediating chloride, sulfate and oxalate transport. Mediates chloride/bicarbonate exchange or chloride-independent bicarbonate extrusion thus assuring bicarbonate secretion. Inhibited by ammonium and thiosulfate
Gene Name:
SLC26A9
Uniprot ID:
Q7LBE3
Molecular weight:
86987.5
General function:
Involved in transporter activity
Specific function:
Acts as a sodium-independent DIDS-sensitive anion exchanger mediating bicarbonate, chloride, sulfate and oxalate transport. May play a role in the maintenance of the electrolyte and acid-base homeostasis in the kidney, by acting as a distal excretory segment-specific anion exchanger. Plays a major role in gastric acid secretion
Gene Name:
SLC26A7
Uniprot ID:
Q8TE54
Molecular weight:
72212.2

Transporters

General function:
Involved in anion transport
Specific function:
Electrogenic sodium/bicarbonate cotransporter with a Na(+):HCO3(-) stoichiometry varying from 1:2 to 1:3. May regulate bicarbonate influx/efflux at the basolateral membrane of cells and regulate intracellular pH
Gene Name:
SLC4A4
Uniprot ID:
Q9Y6R1
Molecular weight:
121459.4
General function:
Involved in inorganic anion exchanger activity
Specific function:
Electrogenic sodium/bicarbonate cotransporter in exchange for intracellular chloride. Plays an important role in regulating intracellular pH
Gene Name:
SLC4A10
Uniprot ID:
Q6U841
Molecular weight:
125945.1
General function:
Involved in anion transport
Specific function:
Electroneutral sodium- and bicarbonate-dependent cotransporter with a Na(+):HCO3(-) 1:1 stoichiometry. Regulates intracellular pH and may play a role in bicarbonate salvage in secretory epithelia. May also have an associated sodium channel activity.
Gene Name:
SLC4A7
Uniprot ID:
Q9Y6M7
Molecular weight:
127358.16
General function:
Involved in anion transport
Specific function:
Mediates sodium- and bicarbonate-dependent electrogenic sodium bicarbonate cotransport, with a Na(+):HCO3(-) stoichiometry of 2:1. May have a housekeeping function in regulating the pH of tissues in which it is expressed. May play a role in mediating Na(+):HCO3(-) cotransport in hepatocytes and intrahepatic cholangiocytes. Also may be important in protecting the renal paranchyma from alterations in urine pH
Gene Name:
SLC4A5
Uniprot ID:
Q9BY07
Molecular weight:
126254.0

Only showing the first 10 proteins. There are 21 proteins in total.