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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:44:18 UTC
HMDB IDHMDB0000595
Secondary Accession Numbers
  • HMDB0003538
  • HMDB0031453
  • HMDB00595
  • HMDB03538
  • HMDB31453
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
Data?1563860658
Synonyms
ValueSource
[CO(OH)2]ChEBI
Dihydrogen carbonateChEBI
H2CO3ChEBI
KoehlensaeureChEBI
Dihydrogen carbonic acidGenerator
Hydrogen carbonic acidGenerator
CarbonateHMDB
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, carbonicHMDB
Carbonic acidHMDB
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
Description belongs to the class of organic compounds known as organic carbonic acids. Organic carbonic acids 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

Industrial application:

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
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-03di-9000000000-310dbbc64fba7d9c667eJSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00du-9300000000-b9ab1da5629a3dfff55fJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-03di-9000000000-53429210d3161a8e792fJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-03di-9000000000-65bbb10c2768f3746b62JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03di-9000000000-b6afca3e3ac002546879JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-03di-9000000000-0ef3797aeb5276c64c90JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-03di-9000000000-5b50453541e6f14e35beJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-03di-9000000000-5b50453541e6f14e35beJSpectraViewer | MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationsNot 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
SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedNormal 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
Hawkinsinuria
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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.
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 IDNot Available
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. Sepai O, Anderson D, Street B, Bird I, Farmer PB, Bailey E: Monitoring of exposure to styrene oxide by GC-MS analysis of phenylhydroxyethyl esters in hemoglobin. Arch Toxicol. 1993;67(1):28-33. [PubMed:8452476 ]
  2. Gross E, Fedotoff O, Pushkin A, Abuladze N, Newman D, Kurtz I: Phosphorylation-induced modulation of pNBC1 function: distinct roles for the amino- and carboxy-termini. J Physiol. 2003 Jun 15;549(Pt 3):673-82. Epub 2003 May 2. [PubMed:12730338 ]
  3. DiGiovanna JJ, Aoyagi T, Taylor JR, Halprin KM: Inhibition of epidermal adenyl cyclase by lithium carbonate. J Invest Dermatol. 1981 Apr;76(4):259-63. [PubMed:6259263 ]
  4. Thakur SC, Thakur SS, Chaube SK, Singh SP: Subchronic supplementation of lithium carbonate induces reproductive system toxicity in male rat. Reprod Toxicol. 2003 Nov-Dec;17(6):683-90. [PubMed:14613820 ]
  5. Faravelli C, Di Bernardo M, Ricca V, Benvenuti P, Bartelli M, Ronchi O: Effects of chronic lithium treatment on the peripheral nervous system. J Clin Psychiatry. 1999 May;60(5):306-10. [PubMed:10362438 ]
  6. Siegel L, Walker SI, Robin NI: Total hydrolyzable glycerol in amniotic fluid, and its relationship to fetal lung maturity. Clin Chem. 1984 Sep;30(9):1546-8. [PubMed:6467568 ]
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  8. Kristensen JM, Kristensen M, Juel C: Expression of Na+/HCO3- co-transporter proteins (NBCs) in rat and human skeletal muscle. Acta Physiol Scand. 2004 Sep;182(1):69-76. [PubMed:15329059 ]
  9. Matousek P, Novotny J, Rudajev V, Svoboda P: Prolonged agonist stimulation does not alter the protein composition of membrane domains in spite of dramatic changes induced in a specific signaling cascade. Cell Biochem Biophys. 2005;42(1):21-40. [PubMed:15673926 ]
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  15. Loiselle FB, Jaschke P, Casey JR: Structural and functional characterization of the human NBC3 sodium/bicarbonate co-transporter carboxyl-terminal cytoplasmic domain. Mol Membr Biol. 2003 Oct-Dec;20(4):307-17. [PubMed:14578046 ]
  16. Antonijevic N, Terzic T, Jovanovic V, Suvajdzic N, Milosevic R, Basara N, Elezovic I: [Acquired amegakaryocytic thrombocytopenia: three case reports and a literature review]. Med Pregl. 2004 May-Jun;57(5-6):292-7. [PubMed:15503803 ]
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Only showing the first 10 proteins. There are 42 proteins in total.

Enzymes

General function:
Involved in acetyl-CoA carboxylase activity
Specific function:
ACC-beta may be involved in the provision of malonyl-CoA or in the regulation of fatty acid oxidation, rather than fatty acid biosynthesis. Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase.
Gene Name:
ACACB
Uniprot ID:
O00763
Molecular weight:
276538.575
Reactions
Adenosine triphosphate + Acetyl-CoA + Hydrogen carbonate → ADP + Phosphate + Malonyl-CoAdetails
Adenosine triphosphate + Holo-[carboxylase] + Hydrogen carbonate → ADP + Phosphate + Carboxybiotin-carboxyl-carrier proteindetails
General function:
Involved in catalytic activity
Specific function:
Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.
Gene Name:
PC
Uniprot ID:
P11498
Molecular weight:
129632.565
Reactions
Adenosine triphosphate + Pyruvic acid + Hydrogen carbonate → ADP + Phosphate + Oxalacetic aciddetails
General function:
Involved in acetyl-CoA carboxylase activity
Specific function:
Catalyzes the rate-limiting reaction in the biogenesis of long-chain fatty acids. Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase.
Gene Name:
ACACA
Uniprot ID:
Q13085
Molecular weight:
269997.01
Reactions
Adenosine triphosphate + Acetyl-CoA + Hydrogen carbonate → ADP + Phosphate + Malonyl-CoAdetails
Adenosine triphosphate + Holo-[carboxylase] + Hydrogen carbonate → ADP + Phosphate + Carboxybiotin-carboxyl-carrier proteindetails
General function:
Involved in oxidoreductase activity
Specific function:
Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.
Gene Name:
ALDH6A1
Uniprot ID:
Q02252
Molecular weight:
57839.31
Reactions
2-Methyl-3-oxopropanoic acid + Coenzyme A + Water + NAD → Propionyl-CoA + Hydrogen carbonate + NADHdetails
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 ligase activity
Specific function:
Not Available
Gene Name:
PCCB
Uniprot ID:
P05166
Molecular weight:
58215.13
Reactions
Adenosine triphosphate + Propionyl-CoA + Hydrogen carbonate → ADP + Phosphate + S-Methylmalonyl-CoAdetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
PCCA
Uniprot ID:
P05165
Molecular weight:
80058.295
Reactions
Adenosine triphosphate + Propionyl-CoA + Hydrogen carbonate → ADP + Phosphate + S-Methylmalonyl-CoAdetails
General function:
Involved in ligase activity
Specific function:
Not Available
Gene Name:
MCCC2
Uniprot ID:
Q9HCC0
Molecular weight:
61332.65
Reactions
Adenosine triphosphate + 3-Methylcrotonyl-CoA + Hydrogen carbonate → ADP + Phosphate + 3-Methylglutaconyl-CoAdetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
MCCC1
Uniprot ID:
Q96RQ3
Molecular weight:
80472.45
Reactions
Adenosine triphosphate + 3-Methylcrotonyl-CoA + Hydrogen carbonate → ADP + Phosphate + 3-Methylglutaconyl-CoAdetails
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

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 42 proteins in total.