Record Information |
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Version | 5.0 |
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Status | Detected and Quantified |
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Creation Date | 2005-11-16 15:48:42 UTC |
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Update Date | 2023-02-21 17:14:58 UTC |
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HMDB ID | HMDB0000595 |
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Secondary Accession Numbers | - HMDB0003538
- HMDB0031453
- HMDB00595
- HMDB03538
- HMDB31453
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Metabolite Identification |
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Common Name | Hydrogen carbonate |
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Description | Bicarbonate, 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. |
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Structure | InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4) |
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Synonyms | Value | Source |
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[CO(OH)2] | ChEBI | Dihydrogen carbonate | ChEBI | H2CO3 | ChEBI | Koehlensaeure | ChEBI | Dihydrogen carbonic acid | Generator | Hydrogen carbonic acid | Generator | Carbonate | Generator, HMDB | Acid OF air | HMDB | Aerial acid | HMDB | Bisodium carbonate | HMDB | Calcined | HMDB | Carbonic acid sodium salt | HMDB | Consal | HMDB | Crystol carbonate | HMDB | Disodium carbonate | HMDB | Mild alkali | HMDB | Na-X | HMDB | Oxyper | HMDB | Sal soda | HMDB | Salt OF soda | HMDB | Scotch soda | HMDB | Soda | HMDB | Soda ash | HMDB | Sodium carbonate | HMDB | Sodium carbonate anhydrous | HMDB | Sodium carbonate hydrated | HMDB | Sodium carbonate peroxyhydrate | HMDB | Solvay soda | HMDB | Trona soda ash | HMDB | Tronalight light soda ash | HMDB | Acid, carbonic | MeSH, HMDB | Carbonic acid | MeSH, HMDB | Ions, bicarbonate | MeSH | Carbonate, hydrogen | MeSH | Carbonates, hydrogen | MeSH | Bicarbonate ion | MeSH | Hydrogen carbonates | MeSH | Bicarbonates | MeSH | Bicarbonate ions | MeSH | Carbonic acid ions | MeSH | Ions, carbonic acid | MeSH |
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Chemical Formula | CH2O3 |
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Average Molecular Weight | 62.0248 |
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Monoisotopic Molecular Weight | 62.00039393 |
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IUPAC Name | carbonic acid |
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Traditional Name | carbonic acid |
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CAS Registry Number | 71-52-3 |
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SMILES | OC(O)=O |
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InChI Identifier | InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4) |
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InChI Key | BVKZGUZCCUSVTD-UHFFFAOYSA-N |
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Chemical Taxonomy |
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Description | Belongs to the class of organic compounds known as organic carbonic acids. Organic carbonic acids are compounds comprising the carbonic acid functional group. |
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Kingdom | Organic compounds |
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Super Class | Organic acids and derivatives |
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Class | Organic carbonic acids and derivatives |
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Sub Class | Organic carbonic acids |
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Direct Parent | Organic carbonic acids |
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Alternative Parents | |
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Substituents | - Carbonic acid
- Organic oxygen compound
- Organic oxide
- Hydrocarbon derivative
- Organooxygen compound
- Carbonyl group
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | |
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Ontology |
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Not Available | Not Available |
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Physical Properties |
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State | Solid |
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Experimental Molecular Properties | Property | Value | Reference |
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Melting Point | 720 °C | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | 12.8 mg/mL (cold water) | MERCK INDEX (1996) | LogP | Not Available | Not Available |
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Experimental Chromatographic Properties | Not Available |
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Predicted Molecular Properties | |
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Predicted Chromatographic Properties | Predicted Collision Cross SectionsPredicted Kovats Retention IndicesUnderivatizedDerivatized |
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Spectra |
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| GC-MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Predicted GC-MS | Predicted GC-MS Spectrum - Hydrogen carbonate GC-MS (Non-derivatized) - 70eV, Positive | splash10-03di-9000000000-310dbbc64fba7d9c667e | 2016-09-22 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Hydrogen carbonate GC-MS (2 TMS) - 70eV, Positive | splash10-00du-9300000000-b9ab1da5629a3dfff55f | 2017-10-06 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Hydrogen carbonate GC-MS (Non-derivatized) - 70eV, Positive | Not Available | 2021-10-12 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Hydrogen carbonate GC-MS (TMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Hydrogen carbonate GC-MS (TBDMS_1_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum | Predicted GC-MS | Predicted GC-MS Spectrum - Hydrogen carbonate GC-MS (TBDMS_2_1) - 70eV, Positive | Not Available | 2021-11-05 | Wishart Lab | View Spectrum |
MS/MS SpectraSpectrum Type | Description | Splash Key | Deposition Date | Source | View |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 10V, Positive-QTOF | splash10-03di-9000000000-53429210d3161a8e792f | 2015-09-14 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 20V, Positive-QTOF | splash10-03di-9000000000-65bbb10c2768f3746b62 | 2015-09-14 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 40V, Positive-QTOF | splash10-03di-9000000000-b6afca3e3ac002546879 | 2015-09-14 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 10V, Negative-QTOF | splash10-03di-9000000000-0ef3797aeb5276c64c90 | 2015-09-15 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 20V, Negative-QTOF | splash10-03di-9000000000-5b50453541e6f14e35be | 2015-09-15 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 40V, Negative-QTOF | splash10-03di-9000000000-5b50453541e6f14e35be | 2015-09-15 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 10V, Negative-QTOF | splash10-03di-9000000000-3142be69389832d3eb4b | 2021-09-21 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 20V, Negative-QTOF | splash10-03di-9000000000-3142be69389832d3eb4b | 2021-09-21 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 40V, Negative-QTOF | splash10-03di-9000000000-3142be69389832d3eb4b | 2021-09-21 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 10V, Positive-QTOF | splash10-03di-9000000000-65a6c4ac46a60ccd0a02 | 2021-09-25 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 20V, Positive-QTOF | splash10-0006-9000000000-75ba60e3edf4ccfcfbe0 | 2021-09-25 | Wishart Lab | View Spectrum | Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - Hydrogen carbonate 40V, Positive-QTOF | splash10-0006-9000000000-a1e091bb1f5fa6e9cbc7 | 2021-09-25 | Wishart Lab | View Spectrum |
NMR SpectraSpectrum Type | Description | Deposition Date | Source | View |
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Predicted 1D NMR | 13C NMR Spectrum (1D, 100 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 1000 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 200 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 300 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 400 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 500 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 600 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 700 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 800 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum | Predicted 1D NMR | 13C NMR Spectrum (1D, 900 MHz, H2O, predicted) | 2022-08-18 | Wishart Lab | View Spectrum |
IR SpectraSpectrum Type | Description | Deposition Date | Source | View |
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Predicted IR Spectrum | IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-) | 2023-02-03 | FELIX lab | View Spectrum | Predicted IR Spectrum | IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+) | 2023-02-03 | FELIX lab | View Spectrum | Predicted IR Spectrum | IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+) | 2023-02-03 | FELIX lab | View Spectrum |
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Biological Properties |
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Cellular Locations | - Cytoplasm
- Extracellular
- Mitochondria
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Biospecimen Locations | - Blood
- Cellular Cytoplasm
- Cerebrospinal Fluid (CSF)
- Saliva
- Urine
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Tissue Locations | Not Available |
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Pathways | |
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Normal Concentrations |
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Blood | Detected and Quantified | 24900.0 +/- 1790.0 uM | Adult (>18 years old) | Both | Normal | | details | Blood | Detected and Quantified | 24700.0 +/- 1200.0 uM | Adult (>18 years old) | Male | Normal | | details | Blood | Detected and Quantified | 23100.0 +/- 1500.0 uM | Adult (>18 years old) | Female | Normal | | details | Blood | Detected and Quantified | 20000.0 (15500.0-22500.0) uM | Newborn (0-30 days old) | Both | Normal | | details | Blood | Detected and Quantified | 20000-30000 uM | Newborn (0-30 days old) | Both | Normal | | details | Blood | Detected and Quantified | 22000-26000 uM | Adult (>18 years old) | Both | Normal | | details | Blood | Detected and Quantified | 23000-26000 uM | Adult (>18 years old) | Not Specified | Normal | | details | Blood | Detected and Quantified | 23000-26000 uM | Infant (0-1 year old) | Not Specified | Normal | | details | Blood | Detected and Quantified | 2950.00721-4425.0108 uM | Newborn (0-30 days old) | Not Specified | Normal | | details | Blood | Detected and Quantified | 24000-32000 uM | Not Specified | Not Specified | Normal | | details | Blood | Detected and Quantified | 22000-29000 uM | Not Specified | Not Specified | Normal | | details | Blood | Detected and Quantified | 22000-29000 uM | Newborn (0-30 days old) | Not Specified | Normal | | details | Blood | Detected and Quantified | 21000-29000 uM | Not Specified | Not Specified | Normal | | details | Blood | Detected and Quantified | >20000 uM | Children (1-13 years old) | Not Specified | Normal | | details | Blood | Detected and Quantified | 24000 (22000-26000) uM | Children (1 - 13 years old) | Both | Normal | | details | Cellular Cytoplasm | Detected and Quantified | 11200 +/- 150 uM | Adult (>18 years old) | Not Specified | Normal | | details | Cerebrospinal Fluid (CSF) | Detected and Quantified | 10000 +/- 1000 uM | Adult (>18 years old) | Both | Normal | | details | Cerebrospinal Fluid (CSF) | Detected and Quantified | 7600 +/- 1600 uM | Adult (>18 years old) | Both | Normal | | details | Saliva | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Not Specified | Normal | | details |
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Abnormal Concentrations |
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Blood | Detected and Quantified | 11000 uM | Newborn (0-30 days old) | Female | 21-hydroxylase deficiency | | details | Blood | Detected and Quantified | 13600.00 uM | Adult (>18 years old) | Both | Diabetic ketoacidosis | | details | Blood | Detected and Quantified | 16200 uM | Infant (0-1 year old) | Male | Dihydrolipoamide Dehydrogenase Deficiency | | details | Blood | Detected and Quantified | 21000 uM | Infant (0-1 year old) | Male | Leigh Syndrome | | details | Blood | Detected and Quantified | 3500 uM | Infant (0-1 year old) | Male | Pyruvate dehydrogenase phosphatase deficiency | | details | Blood | Detected and Quantified | 17000-18000 uM | Children (1-13 years old) | Female | Fanconi Bickel syndrome | | details | Blood | Detected and Quantified | 20100 uM | Adolescent (13-18 years old) | Both | Renal tubular acidosis, distal, RTA type 1 | | details | Blood | Detected and Quantified | 17800-19500 uM | Adult (>18 years old) | Both | Renal tubular acidosis, distal, RTA type 1 | | details | Blood | Detected and Quantified | 4800-15000 uM | Infant (0-1 year old) | Both | Renal tubular acidosis, distal, RTA type 1 | | details | Blood | Detected and Quantified | 5500-13000 uM | Newborn (0-30 days old) | Both | Renal tubular acidosis, distal, RTA type 1 | | details | Blood | Detected and Quantified | 6300-19500 uM | Children (1-13 years old) | Both | Renal tubular acidosis, distal, RTA type 1 | | details | Blood | Detected and Quantified | 16900 uM | Newborn (0-30 days old) | Male | 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency | | details | Blood | Detected and Quantified | 30000-43000 uM | Infant (0-1 year old) | Both | Congenital chloride diarrhea | | details | Blood | Detected and Quantified | 28000-35000 uM | Adult (>18 years old) | Both | Gitelman syndrome | | details | Blood | Detected and Quantified | 28000-33000 uM | Adult (>18 years old) | Female | Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES) | | details | Blood | Detected and Quantified | 12800 uM | Adult (>18 years old) | Female | Fanconi syndrome | | details | Blood | Detected and Quantified | 5000-15000 uM | Children (1-13 years old) | Male | Monocarboxylate transporter 1 deficiency | | details | Blood | Detected and Quantified | 7000-13000 uM | Infant (0-1 year old) | Male | Carnitine palmitoyltransferase deficiency I | | details | Blood | Detected and Quantified | 22400 +/- 700 uM | Children (1-13 years old) | Male | Fanconi syndrome | | details | Blood | Detected and Quantified | 12700 uM | Adolescent (13-18 years old) | Female | Renal tubular acidosis, proximal, with ocular abnormalities and mental retardation | | details | Blood | Detected and Quantified | 9300 uM | Newborn (0-30 days old) | Male | Succinyl CoA: 3-ketoacid CoA transferase deficiency | | details | Blood | Detected and Quantified | 9700-16600 uM | Infant (0-1 year old) | Both | Congenital secretory diarrhea | | details | Blood | Detected and Quantified | 2130.561 uM | Newborn (0-30 days old) | Not Available | Pseudohypoaldosteronism, type I, autosomal dominant | | details | Blood | Detected and Quantified | 15000 uM | Newborn (0-30 days old) | Male | 2-Ketoglutarate dehydrogenase complex deficiency | | details | Blood | Detected and Quantified | 11000-21000 uM | Children (1-13 years old) | Both | 2-Ketoglutarate dehydrogenase complex deficiency | | details | Blood | Detected and Quantified | 18000 uM | Newborn (0-30 days old) | Female | Bartter Syndrome, Type 2, Antenatal | | details | Blood | Detected and Quantified | 25500.00 (24000.00-27000.00) uM | Adult (>18 years old) | Both | Hawkinsinuria | | details | Blood | Detected and Quantified | 12000.00 (10000.00-14000.00) uM | Children (1-13 years old) | Both | Hawkinsinuria | | details | Blood | Detected and Quantified | 19800-28300 uM | Newborn (0-30 days old) | Female | Bartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness | | details | Blood | Detected and Quantified | 23900 uM | Infant (0-1 year old) | Female | Bartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness | | details | Blood | Detected and Quantified | 25900-37200 uM | Children (1-13 years old) | Female | Bartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness | | details | Blood | Detected and Quantified | 33500 uM | Infant (0-1 year old) | Not Specified | Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness | | details | Blood | Detected and Quantified | 24000-38000 uM | Newborn (0-30 days old) | Male | Bartter Syndrome, Type 5, Antenatal, Transient | | details | Blood | Detected and Quantified | 38000 uM | Newborn (0-30 days old) | Female | Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness | | details | Blood | Detected and Quantified | 18900 +/- 2500 uM | Adolescent (13-18 years old) | Both | Leigh Syndrome, French Canadian Type | | details | Blood | Detected and Quantified | 16000 uM | Children (1-13 years old) | Male | Lipoyltransferase 1 Deficiency | | details | Blood | Detected and Quantified | 11000-15000 uM | Newborn (0-30 days old) | Male | Narp Syndrome | | details | Blood | Detected and Quantified | 23000 uM | Adult (>18 years old) | Male | Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness | | details | Urine | Detected and Quantified | 0 umol/mmol creatinine | Children (1-13 years old) | Male | Fanconi syndrome | | details | Urine | Detected and Quantified | 3060 umol/mmol creatinine | Adolescent (13-18 years old) | Female | Renal tubular acidosis, proximal, with ocular abnormalities and mental retardation | | details |
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Associated Disorders and Diseases |
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Disease References | Hawkinsinuria |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 ]
- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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) |
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- 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 |
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- Cheng HM, Jap TS, Ho LT: Fanconi syndrome: report of a case. J Formos Med Assoc. 1990 Dec;89(12):1115-7. [PubMed:1982686 ]
- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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 |
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- 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.
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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)
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External Links |
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DrugBank ID | Not Available |
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Phenol Explorer Compound ID | Not Available |
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FooDB ID | FDB023191 |
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KNApSAcK ID | Not Available |
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Chemspider ID | 747 |
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KEGG Compound ID | C01353 |
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BioCyc ID | Not Available |
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BiGG ID | 1436647 |
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Wikipedia Link | Carbonic acid |
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METLIN ID | 6944 |
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PubChem Compound | 767 |
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PDB ID | Not Available |
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ChEBI ID | 28976 |
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Food Biomarker Ontology | Not Available |
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VMH ID | HCO3 |
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MarkerDB ID | MDB00013430 |
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Good Scents ID | Not Available |
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References |
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Synthesis Reference | Nakajima, Fumiaki; Arima, Toshikazu; Kikuchi, Shintaro; Hirano, Hachiro. Production of alkaline hydrogen carbonate. Jpn. Kokai Tokkyo Koho (2005), 15 pp. |
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Material Safety Data Sheet (MSDS) | Download (PDF) |
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General References | - 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 ]
- 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 ]
- Severina IS, Pyatakova NV, Shchegolev AY, Ponomarev GV: YC-1-like potentiation of NO-dependent activation of soluble guanylate cyclase by derivatives of protoporphyrin IX. Biochemistry (Mosc). 2006 Mar;71(3):340-4. [PubMed:16545073 ]
- Bomhard EM, Brendler-Schwaab SY, Freyberger A, Herbold BA, Leser KH, Richter M: O-phenylphenol and its sodium and potassium salts: a toxicological assessment. Crit Rev Toxicol. 2002;32(6):551-625. [PubMed:12487365 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- Bartsch I, Zschaler I, Haseloff M, Steinberg P: Establishment of a long-term culture system for rat colon epithelial cells. In Vitro Cell Dev Biol Anim. 2004 Sep-Oct;40(8-9):278-84. [PubMed:15723563 ]
- 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 ]
- Xu W, Yoon SI, Huang P, Wang Y, Chen C, Chong PL, Liu-Chen LY: Localization of the kappa opioid receptor in lipid rafts. J Pharmacol Exp Ther. 2006 Jun;317(3):1295-306. Epub 2006 Feb 27. [PubMed:16505160 ]
- Perrild H, Hegedus L, Arnung K: Sex related goitrogenic effect of lithium carbonate in healthy young subjects. Acta Endocrinol (Copenh). 1984 Jun;106(2):203-8. [PubMed:6428121 ]
- Sack DA, Sack RB, Nair GB, Siddique AK: Cholera. Lancet. 2004 Jan 17;363(9404):223-33. [PubMed:14738797 ]
- 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 ]
- 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 ]
- Chang TC: Influence of lithium carbonate on the thyrotropin receptor in vitro. Taiwan Yi Xue Hui Za Zhi. 1989 Jan;88(1):13-7. [PubMed:2547015 ]
- Arthurs CE, Jarvis GN, Russell JB: The effect of various carbonate sources on the survival of Escherichia coli in dairy cattle manure. Curr Microbiol. 2001 Sep;43(3):220-4. [PubMed:11400074 ]
- Piepho RW, Culbertson VL, Rhodes RS: Drug interactions with the calcium-entry blockers. Circulation. 1987 Jun;75(6 Pt 2):V181-94. [PubMed:2882874 ]
- Saran AS: Antidiabetic effects of lithium. J Clin Psychiatry. 1982 Sep;43(9):383-4. [PubMed:6811566 ]
- Amsterdam JD, Maislin G, Rybakowski J: A possible antiviral action of lithium carbonate in herpes simplex virus infections. Biol Psychiatry. 1990 Feb 15;27(4):447-53. [PubMed:2155671 ]
- Gultekin I, Ince NH: Ultrasonic destruction of bisphenol-A: the operating parameters. Ultrason Sonochem. 2008 Apr;15(4):524-9. Epub 2007 Jun 10. [PubMed:17644462 ]
- 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 ]
- Casey JR: Why bicarbonate? Biochem Cell Biol. 2006 Dec;84(6):930-9. [PubMed:17215880 ]
- (). Duke, James A. (1992) Handbook of phytochemical constituents of GRAS herbs and other economic plants. Boca Raton, FL. CRC Press.. .
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