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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-08-15 22:06:05 UTC
Update Date2018-05-20 19:04:15 UTC
HMDB IDHMDB0000586
Secondary Accession Numbers
  • HMDB00586
Metabolite Identification
Common NamePotassium
DescriptionPotassium is an essential electrolyte. Potassium balance is crucial for regulating the excitability of nerves and muscles and so critical for regulating contractility of cardiac muscle. Although the most important changes seen in the presence of deranged potassium are cardiac, smooth muscle is also affected with increasing muscle weakness, a feature of both hyperkalaemia and hypokalaemia. Physiologically, it exists as an ion in the body. Potassium (K+) is a positively charged electrolyte, cation, which is present throughout the body in both intracellular and extracellular fluids. The majority of body potassium, >90%, are intracellular. It moves freely from intracellular fluid (ICF) to extracellular fluid (ECF) and vice versa when adenosine triphosphate increases the permeability of the cell membrane. It is mainly replaced inside or outside the cells by another cation, sodium (Na+). The movement of potassium into or out of the cells is linked to certain body hormones and also to certain physiological states. Standard laboratory tests measure ECF potassium. Potassium enters the body rapidly during food ingestion. Insulin is produced when a meal is eaten; this causes the temporary movement of potassium from ECF to ICF. Over the ensuing hours, the kidneys excrete the ingested potassium and homeostasis is returned. In the critically ill patient, suffering from hyperkalaemia, this mechanism can be manipulated beneficially by administering high concentration (50%) intravenous glucose. Insulin can be added to the glucose, but glucose alone will stimulate insulin production and cause movement of potassium from ECF to ICF. The stimulation of alpha receptors causes increased movement of potassium from ICF to ECF. A noradrenaline infusion can elevate serum potassium levels. An adrenaline infusion, or elevated adrenaline levels, can lower serum potassium levels. Metabolic acidosis causes a rise in extracellular potassium levels. In this situation, excess of hydrogen ions (H+) are exchanged for intracellular potassium ions, probably as a result of the cellular response to a falling blood pH. Metabolic alkalosis causes the opposite effect, with potassium moving into the cells. (PMID: 17883675 ).
Structure
Thumb
Synonyms
ValueSource
K(+)ChEBI
K+ChEBI
POTASSIUM ionChEBI
KaliumHMDB
Potassium (ion)HMDB
Potassium (k+)HMDB
Potassium cationHMDB
Potassium ion (k+)HMDB
Potassium ion (K1+)HMDB
Potassium ion(+)HMDB
Potassium ion(1+)HMDB
Potassium monocationHMDB
Potassium(+)HMDB
Potassium(1+)HMDB
Potassium(1+) ionHMDB
Potassium(I) cationHMDB
Chemical FormulaK
Average Molecular Weight39.0983
Monoisotopic Molecular Weight38.963706861
IUPAC Namepotassium(1+) ion
Traditional Namepotassium(1+) ion
CAS Registry Number7440-09-7
SMILES
[K+]
InChI Identifier
InChI=1S/K/q+1
InChI KeyNPYPAHLBTDXSSS-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of inorganic compounds known as homogeneous alkali metal compounds. These are inorganic compounds containing only metal atoms,with the largest atom being a alkali metal atom.
KingdomInorganic compounds
Super ClassHomogeneous metal compounds
ClassHomogeneous alkali metal compounds
Sub ClassNot Available
Direct ParentHomogeneous alkali metal compounds
Alternative ParentsNot Available
Substituents
  • Homogeneous alkali metal
Molecular FrameworkNot Available
External Descriptors
Ontology
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point63.2 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
logP0.2ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count0ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area0 ŲChemAxon
Rotatable Bond Count0ChemAxon
Refractivity0 m³·mol⁻¹ChemAxon
Polarizability1.78 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-9000000000-560fc6f738e9570ec8a2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-9000000000-560fc6f738e9570ec8a2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-9000000000-560fc6f738e9570ec8a2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-9000000000-bcbe5ea7d5d32f6a9598View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-000i-9000000000-bcbe5ea7d5d32f6a9598View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-000i-9000000000-bcbe5ea7d5d32f6a9598View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Golgi apparatus
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3500-5000 uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified4600.0 +/- 500.0 uMChildren (1-13 years old)Both
Normal
    • Geigy Scientific ...
details
BloodDetected and Quantified4200.0 (3600.0-4800.0) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified4100.0 (3600.0-4600.0) uMAdult (>18 years old)Female
Normal
    • Geigy Scientific ...
details
BloodDetected and Quantified3500-4700 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified3700-5900 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified3500-5100 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified3500-5000 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified3500-5100 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified3300-4900 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified4000-6400 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified3500-5500 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified3500-5100 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified3500-5300 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified4000-6500 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified3300-5100 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified3300-5100 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified3500-5000 uMAdult (>18 years old)Female
Normal
details
BloodDetected and Quantified3500-5000 uMAdult (>18 years old)Male
Normal
details
BloodDetected and Quantified4560.0 +/- 560.0 uMNewborn (0-30 days old)Not Specified
Normal
    • Geigy Scientific ...
details
BloodDetected and Quantified3500-5000 uMNot SpecifiedNot Specified
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified2960.0 (2620.0-3300.0) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified2163 +/- 274 uMAdult (>18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified24800 +/- 9900 uMNot SpecifiedBoth
Normal
details
SalivaDetected and Quantified21830 +/- 2080 uMAdult (>18 years old)Not Specified
Normal
details
SalivaDetected and Quantified20250 +/- 2770 uMAdult (>18 years old)Not Specified
Normal
details
SalivaDetected and Quantified19009.26 +/- 7828.18 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
UrineDetected and Quantified3593.14 (553.33-8078.58) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2133.333 +/- 1400 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified2893.333 +/- 1446.667 umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified3180 +/- 1093.333 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
UrineDetected and Quantified3586.667 +/- 933.333 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3593.333 +/- 1226.667 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
UrineDetected and Quantified2773.333 +/- 1166.667 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified4600 +/- 1066.667 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified56800 +/- 1666.667 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified2926.667 +/- 1133.333 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified5466.667 +/- 1066.667 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified4605.2 (2631.5 - 6578.9) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified4407.8 +/- 131.5 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified5460.00 +/- 197.00 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified6000-8000 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified6900 uMNewborn (0-30 days old)MaleAdrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency details
BloodDetected and Quantified7800 uMAdult (>18 years old)Female
21-hydroxylase deficiency
details
BloodDetected and Quantified7100-9000 uMNewborn (0-30 days old)Female
21-hydroxylase deficiency
details
BloodDetected and Quantified2600 (2300-2900) uMChildren (1-13 years old)Both
Bartter Syndrome, Type 3
details
BloodDetected and Quantified2700 uMInfant (0-1 year old)Female
Apparent mineralocorticoid excess
details
BloodDetected and Quantified3400 uMAdolescent (13-18 years old)Both
Renal tubular acidosis, distal, RTA type 1
details
BloodDetected and Quantified1580-4500 uMChildren (1-13 years old)BothRenal tubular acidosis, distal, RTA type 1 details
BloodDetected and Quantified2100-3200 uMInfant (0-1 year old)BothRenal tubular acidosis, distal, RTA type 1 details
BloodDetected and Quantified2200-4500 uMNewborn (0-30 days old)Both
Renal tubular acidosis, distal, RTA type 1
details
BloodDetected and Quantified3300-4600 uMAdult (>18 years old)Both
Renal tubular acidosis, distal, RTA type 1
details
BloodDetected and Quantified3700-4300 uMAdult (>18 years old)Male
Primary Hypomagnesemia
details
BloodDetected and Quantified86000-87000 uMNewborn (0-30 days old)FemaleBartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness details
BloodDetected and Quantified101000 uMInfant (0-1 year old)FemaleBartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness details
BloodDetected and Quantified81000-97000 uMChildren (1-13 years old)FemaleBartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness details
BloodDetected and Quantified2800 uMNewborn (0-30 days old)MaleDonohue Syndrome details
BloodDetected and Quantified6900 uMInfant (0-1 year old)FemaleLipoid Adrenal Hyperplasia details
BloodDetected and Quantified8200 uMNewborn (0-30 days old)Not AvailablePseudohypoaldosteronism, type I, autosomal dominant details
BloodDetected and Quantified1800-2800 uMInfant (0-1 year old)BothCongenital chloride diarrhea details
BloodDetected and Quantified1600 uMAdult (>18 years old)FemaleFanconi syndrome details
BloodDetected and Quantified1600-3000 uMAdult (>18 years old)BothGitelman syndrome details
BloodDetected and Quantified2900-3150 uMAdult (>18 years old)FemaleSeizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES) details
BloodDetected and Quantified2900 (2500-4000) uMChildren (1-13 years old)BothCongenital chloride diarrhea details
BloodDetected and Quantified4800-5800 uMInfant (0-1 year old)BothCongenital secretory diarrhea details
BloodDetected and Quantified5100 uMInfant (0-1 year old)FemaleCorticosterone methyloxidase I deficiency- CMO I details
BloodDetected and Quantified3400 uMChildren (1-13 years old)MaleFanconi syndrome details
BloodDetected and Quantified3300-3600 uMAdult (>18 years old)MaleGlucocorticoid resistance details
BloodDetected and Quantified2600-6800 uMNewborn (0-30 days old)FemaleBartter Syndrome, Type 2, Antenatal details
BloodDetected and Quantified2800 uMAdolescent (13-18 years old)FemaleAdrenal hyperplasia, congenital, due to 17-alpha-hydroxylase deficiency details
BloodDetected and Quantified2800-3300 uMNewborn (0-30 days old)MaleBartter Syndrome, Type 5, Antenatal, Transient details
BloodDetected and Quantified6900 uMNewborn (0-30 days old)FemaleLipoid Adrenal Hyperplasia details
BloodDetected and Quantified4300-4600 uMInfant (0-1 year old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified3300-4400 uMChildren (1-13 years old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified3400-5900 uMAdolescent (13-18 years old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified3200-3800 uMAdult (>18 years old)Not SpecifiedOculocerebrorenal Syndrome of Lowe details
BloodDetected and Quantified2800 uMNewborn (0-30 days old)Female
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
details
BloodDetected and Quantified2900-3500 uMInfant (0-1 year old)Not Specified
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
details
BloodDetected and Quantified2400-2700 uMChildren (1-13 years old)Female
Bartter Syndrome, Type 1, Antenatal
details
BloodDetected and Quantified2000 uMAdult (>18 years old)Male
Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness
details
BloodDetected and Quantified2800-2900 uMNewborn (0-30 days old)Female
Bartter Syndrome, Type 1, Antenatal
details
BloodDetected and Quantified6100 uMNewborn (0-30 days old)Female
Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete
details
BloodDetected and Quantified7100 uMNewborn (0-30 days old)Female
Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete
details
UrineDetected and Quantified2097.278-3273.800 umol/mmol creatinineAdult (>18 years old)Male
Primary Hypomagnesemia
details
UrineDetected and Quantified16180-38180 umol/mmol creatinineAdult (>18 years old)FemaleSeizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES) 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 ]
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
  1. Guven A, Polat S: Testicular Adrenal Rest Tumor in Two Brothers with a Novel Mutation in the 3-Beta-Hydroxysteroid Dehydrogenase-2 Gene. J Clin Res Pediatr Endocrinol. 2017 Mar 1;9(1):85-90. doi: 10.4274/jcrpe.3306. Epub 2016 Jul 29. [PubMed:27476613 ]
Corticosterone methyl oxidase I deficiency
  1. Ustyol A, Atabek ME, Taylor N, Yeung MC, Chan AO: Corticosterone Methyl Oxidase Deficiency Type 1 with Normokalemia in an Infant. J Clin Res Pediatr Endocrinol. 2016 Sep 1;8(3):356-9. doi: 10.4274/jcrpe.2824. Epub 2016 Apr 29. [PubMed:27125267 ]
Oculocerebrorenal syndrome
  1. Charnas LR, Bernardini I, Rader D, Hoeg JM, Gahl WA: Clinical and laboratory findings in the oculocerebrorenal syndrome of Lowe, with special reference to growth and renal function. N Engl J Med. 1991 May 9;324(19):1318-25. doi: 10.1056/NEJM199105093241904. [PubMed:2017228 ]
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 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Primary hypomagnesemia
  1. Jin-no Y, Kamiya Y, Okada M, Hirako M, Takada N, Kawaguchi M: Primary hypomagnesemia caused by isolated magnesium malabsorption: atypical case in adult. Intern Med. 1999 Mar;38(3):261-5. [PubMed:10337938 ]
Congenital Adrenal Hyperplasia, due to 17-Hydroxylase-Deficiency
  1. Wong SL, Shu SG, Tsai CR: Seventeen alpha-hydroxylase deficiency. J Formos Med Assoc. 2006 Feb;105(2):177-81. doi: 10.1016/S0929-6646(09)60342-9. [PubMed:16477341 ]
Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete
  1. Kim CJ, Lin L, Huang N, Quigley CA, AvRuskin TW, Achermann JC, Miller WL: Severe combined adrenal and gonadal deficiency caused by novel mutations in the cholesterol side chain cleavage enzyme, P450scc. J Clin Endocrinol Metab. 2008 Mar;93(3):696-702. doi: 10.1210/jc.2007-2330. Epub 2008 Jan 8. [PubMed:18182448 ]
Apparent mineralocorticoid excess
  1. New MI, Levine LS, Biglieri EG, Pareira J, Ulick S: Evidence for an unidentified steroid in a child with apparent mineralocorticoid hypertension. J Clin Endocrinol Metab. 1977 May;44(5):924-33. doi: 10.1210/jcem-44-5-924. [PubMed:870517 ]
Bartter Syndrome, Type 1, Antenatal
  1. Adachi M, Asakura Y, Sato Y, Tajima T, Nakajima T, Yamamoto T, Fujieda K: Novel SLC12A1 (NKCC2) mutations in two families with Bartter syndrome type 1. Endocr J. 2007 Dec;54(6):1003-7. Epub 2007 Nov 12. [PubMed:17998760 ]
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 3
  1. Seys E, Andrini O, Keck M, Mansour-Hendili L, Courand PY, Simian C, Deschenes G, Kwon T, Bertholet-Thomas A, Bobrie G, Borde JS, Bourdat-Michel G, Decramer S, Cailliez M, Krug P, Cozette P, Delbet JD, Dubourg L, Chaveau D, Fila M, Jourde-Chiche N, Knebelmann B, Lavocat MP, Lemoine S, Djeddi D, Llanas B, Louillet F, Merieau E, Mileva M, Mota-Vieira L, Mousson C, Nobili F, Novo R, Roussey-Kesler G, Vrillon I, Walsh SB, Teulon J, Blanchard A, Vargas-Poussou R: Clinical and Genetic Spectrum of Bartter Syndrome Type 3. J Am Soc Nephrol. 2017 Aug;28(8):2540-2552. doi: 10.1681/ASN.2016101057. Epub 2017 Apr 5. [PubMed:28381550 ]
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 ]
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 ]
  2. Lubani MM, Doudin KI, Sharda DC, Shaltout AA, al-Shab TS, Abdul Al YK, Said MA, Salhi MM, Ahmed SA: Congenital chloride diarrhoea in Kuwaiti children. Eur J Pediatr. 1989 Jan;148(4):333-6. [PubMed:2651131 ]
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 ]
Donohue Syndrome
  1. Nijim Y, Awni Y, Adawi A, Bowirrat A: Classic Case Report of Donohue Syndrome (Leprechaunism; OMIM *246200): The Impact of Consanguineous Mating. Medicine (Baltimore). 2016 Feb;95(6):e2710. doi: 10.1097/MD.0000000000002710. [PubMed:26871809 ]
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 ]
Glucocorticoid resistance
  1. Donner KM, Hiltunen TP, Janne OA, Sane T, Kontula K: Generalized glucocorticoid resistance caused by a novel two-nucleotide deletion in the hormone-binding domain of the glucocorticoid receptor gene NR3C1. Eur J Endocrinol. 2012 Dec 10;168(1):K9-K18. doi: 10.1530/EJE-12-0532. Print 2013 Jan. [PubMed:23076843 ]
Lipoid Congenital Adrenal Hyperplasia
  1. Fujieda K, Tajima T, Nakae J, Sageshima S, Tachibana K, Suwa S, Sugawara T, Strauss JF 3rd: Spontaneous puberty in 46,XX subjects with congenital lipoid adrenal hyperplasia. Ovarian steroidogenesis is spared to some extent despite inactivating mutations in the steroidogenic acute regulatory protein (StAR) gene. J Clin Invest. 1997 Mar 15;99(6):1265-71. doi: 10.1172/JCI119284. [PubMed:9077535 ]
  2. Hauffa BP, Miller WL, Grumbach MM, Conte FA, Kaplan SL: Congenital adrenal hyperplasia due to deficient cholesterol side-chain cleavage activity (20, 22-desmolase) in a patient treated for 18 years. Clin Endocrinol (Oxf). 1985 Nov;23(5):481-93. [PubMed:3841304 ]
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 ]
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
  • 179800 (Renal tubular acidosis, distal, RTA type 1)
  • 248250 (Primary hypomagnesemia)
  • 218030 (Apparent mineralocorticoid excess)
  • 201910 (21-Hydroxylase deficiency)
  • 202110 (Congenital Adrenal Hyperplasia, due to 17-Hydroxylase-Deficiency)
  • 602522 (Bartter Syndrome, Type 4A, Neonatal, with Sensorineural Deafness)
  • 601678 (Bartter Syndrome, Type 1, Antenatal)
  • 613743 (Adrenal insufficiency, congenital, with 46,XY sex reversal, partial or complete)
  • 241200 (Bartter Syndrome, Type 2, Antenatal)
  • 201810 (Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency)
  • 607364 (Bartter Syndrome, Type 3)
  • 613090 (Bartter Syndrome, Type 4B, Neonatal, With Sensorineural Deafness)
  • 246200 (Donohue Syndrome)
  • 201710 (Lipoid Congenital Adrenal Hyperplasia)
  • 177735 (Pseudohypoaldosteronism, type I, autosomal dominant)
  • 214700 (Congenital chloride diarrhea)
  • 263800 (Gitelman syndrome)
  • 612780 (Seizures, sensorineural deafness, ataxia, mental retardation, and electrolyte imbalance (SESAMES))
  • 270420 (Congenital secretory diarrhea)
  • 203400 (Corticosterone methyl oxidase I deficiency)
  • 615962 (Glucocorticoid resistance)
  • 300971 (Bartter Syndrome, Type 5, Antenatal, Transient)
  • 309000 (Oculocerebrorenal syndrome)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB003521
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDC00238
BioCyc IDK%2b
BiGG ID34349
Wikipedia LinkPotassium
METLIN ID3197
PubChem Compound813
PDB IDNot Available
ChEBI ID29103
References
Synthesis ReferenceAlberti, Augusto. Recovering potassium salts from the refuse liquor of the manufacture of tartaric acid. (1910), US 957295 19100510 CAN 4:13164 AN 1910:13164
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Schaafsma A, de Vries PJ, Saris WH: Delay of natural bone loss by higher intakes of specific minerals and vitamins. Crit Rev Food Sci Nutr. 2001 May;41(4):225-49. [PubMed:11401244 ]
  2. Preuss HG: Diet, genetics and hypertension. J Am Coll Nutr. 1997 Aug;16(4):296-305. [PubMed:9263178 ]
  3. Beede DK: Mineral and water nutrition. Vet Clin North Am Food Anim Pract. 1991 Jul;7(2):373-90. [PubMed:1893277 ]
  4. Brooks G: Potassium additive algorithm for use in continuous renal replacement therapy. Nurs Crit Care. 2006 Nov-Dec;11(6):273-80. [PubMed:17883675 ]

Only showing the first 10 proteins. There are 55 proteins in total.

Enzymes

General function:
Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHA
Uniprot ID:
P12694
Molecular weight:
50470.58
General function:
Involved in methionine adenosyltransferase activity
Specific function:
Catalyzes the formation of S-adenosylmethionine from methionine and ATP.
Gene Name:
MAT2A
Uniprot ID:
P31153
Molecular weight:
43660.37
General function:
Involved in methionine adenosyltransferase activity
Specific function:
Catalyzes the formation of S-adenosylmethionine from methionine and ATP.
Gene Name:
MAT1A
Uniprot ID:
Q00266
Molecular weight:
43647.6
General function:
Involved in magnesium ion binding
Specific function:
Glycolytic enzyme that catalyzes the transfer of a phosphoryl group from phosphoenolpyruvate (PEP) to ADP, generating ATP. Stimulates POU5F1-mediated transcriptional activation. Plays a general role in caspase independent cell death of tumor cells. The ratio betwween the highly active tetrameric form and nearly inactive dimeric form determines whether glucose carbons are channeled to biosynthetic processes or used for glycolytic ATP production. The transition between the 2 forms contributes to the control of glycolysis and is important for tumor cell proliferation and survival.
Gene Name:
PKM
Uniprot ID:
P14618
Molecular weight:
65930.14
General function:
Involved in magnesium ion binding
Specific function:
Plays a key role in glycolysis (By similarity).
Gene Name:
PKLR
Uniprot ID:
P30613
Molecular weight:
61829.575
General function:
Involved in catalytic activity
Specific function:
Catalyzes the conversion of inosine 5'-phosphate (IMP) to xanthosine 5'-phosphate (XMP), the first committed and rate-limiting step in the de novo synthesis of guanine nucleotides, and therefore plays an important role in the regulation of cell growth. Could also have a single-stranded nucleic acid-binding activity and could play a role in RNA and/or DNA metabolism. It may also have a role in the development of malignancy and the growth progression of some tumors.
Gene Name:
IMPDH2
Uniprot ID:
P12268
Molecular weight:
55804.495
General function:
Involved in catalytic activity
Specific function:
Catalyzes the conversion of inosine 5'-phosphate (IMP) to xanthosine 5'-phosphate (XMP), the first committed and rate-limiting step in the de novo synthesis of guanine nucleotides, and therefore plays an important role in the regulation of cell growth. Could also have a single-stranded nucleic acid-binding activity and could play a role in RNA and/or DNA metabolism. It may also have a role in the development of malignancy and the growth progression of some tumors.
Gene Name:
IMPDH1
Uniprot ID:
P20839
Molecular weight:
63252.24
General function:
Involved in pyridoxal kinase activity
Specific function:
Required for synthesis of pyridoxal-5-phosphate from vitamin B6.
Gene Name:
PDXK
Uniprot ID:
O00764
Molecular weight:
35102.105
General function:
Involved in sodium:potassium-exchanging ATPase activity
Specific function:
This is the non-catalytic component of the active enzyme, which catalyzes the hydrolysis of ATP coupled with the exchange of Na(+) and K(+) ions across the plasma membrane. The exact function of the beta-3 subunit is not known
Gene Name:
ATP1B3
Uniprot ID:
P54709
Molecular weight:
31512.3
General function:
Involved in sodium:potassium-exchanging ATPase activity
Specific function:
This is the non-catalytic component of the active enzyme, which catalyzes the hydrolysis of ATP coupled with the exchange of Na(+) and K(+) ions across the plasma membrane. The exact function of the beta-2 subunit is not known
Gene Name:
ATP1B2
Uniprot ID:
P14415
Molecular weight:
33366.9

Only showing the first 10 proteins. There are 55 proteins in total.