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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 08:04:59 UTC
HMDB IDHMDB0000240
Secondary Accession Numbers
  • HMDB00240
Metabolite Identification
Common NameSulfite
DescriptionEndogenous sulfite is generated as a consequence of the body's normal processing of sulfur-containing amino acids. Sulfites occur as a consequence of fermentation and also occur naturally in a number of foods and beverages. As food additives, sulfiting agents were first used in 1664 and have been approved in the United States since the 1800s. Sulfite is an allergen, a neurotoxin, and a metabotoxin. An allergen is a compound that causes allergic reactions such as wheezing, rash, or rhinitis. A neurotoxin is a substance that causes damage to nerves or brain tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. As an allergen, sulfite is known to induce asthmatic reactions. Sulfite sensitivity occurs most often in asthmatic adults (predominantly women), but it is also occasionally reported in preschool children. Adverse reactions to sulfites in nonasthmatics are extremely rare. Asthmatics who are steroid-dependent or who have a higher degree of airway hyperreactivity may be at greater risk of experiencing a reaction to sulfite-containing foods. Sulfite sensitivity reactions vary widely, ranging from no reaction to severe. The majority of reactions are mild. These manifestations may include dermatologic, respiratory, or gastrointestinal signs and symptoms. The precise mechanisms of the sensitivity responses have not been completely elucidated: inhalation of sulfur dioxide (SO2) generated in the stomach following ingestion of sulfite-containing foods or beverages, a deficiency in a mitochondrial enzyme, and an IgE-mediated immune response have all been implicated. Exogenously supplied sulfite is detoxified by the enzyme sulfite oxidase. Sulfite oxidase (EC 1.8.3.1) is 1 of 3 enzymes in humans that require molybdenum as a cofactor. Under certain circumstances, chronically high levels of sulfite can lead to serious neurotoxicity. Sulfite oxidase deficiency (also called molybdenum cofactor deficiency) is a rare autosomal inherited disease that is typified by high concentrations of sulfite in the blood and urine. It is characterized by severe neurological symptoms such as untreatable seizures, attenuated growth of the brain, and mental retardation. It results from defects in the enzyme sulfite oxidase, which is responsible for the oxidation of sulfite to sulfate. This sulfite to sulfate reaction is the final step in the degradation of sulfur-containing metabolites (including the amino acids cysteine and methionine). The term "isolated sulfite oxidase deficiency" is used to define the deficiency caused by mutations in the sulfite oxidase gene. This differentiates it from another version of sulfite oxidase deficiency that is due to defects in the molybdenum cofactor biosynthetic pathway (with mutations in the MOCS1 or MOCS2 genes). Isolated sulfite oxidase deficiency is a rare but devastating neurologic disease that usually presents in early infancy with seizures and alterations in muscle tone (PMID: 16234925 , 16140720 , 8586770 ). Sulfite oxidase deficiency (as caused by MOCS1 or MOCS2) may be treated with cPMP, a precursor of the molybdenum cofactor (PMID: 20385644 ). The mechanism behind sulfite neurotoxicity appears to be related to its ability to bind and inhibit glutamate dehydrogenase (GDH). Inhibition of GDH leads to a decrease in alpha-ketoglutarate and a diminished flux through the tricarboxylic acid cycle. This is accompanied by a decrease in NADH through the mitochondrial electron transport chain, which leads to a decrease in mitochondrial membrane potential and in ATP synthesis. Since glutamate is a major metabolite in the brain, inhibition of GDH by sulfite appears to contribute to neural damage characteristic of sulfite oxidase deficiency in human infants (PMID: 15273247 ).
Structure
Thumb
Synonyms
ValueSource
[so(OH)2]ChEBI
Acide sulfureuxChEBI
acido SulfurosoChEBI
H2SO3ChEBI
S(O)(OH)2ChEBI
Schweflige saeureChEBI
SulfiteChEBI
Sulphurous acidChEBI
Acide sulphureuxGenerator
acido SulphurosoGenerator
SulphiteGenerator
Sulfur dioxide solutionHMDB
Sulfuric(IV) acid (H2SO3)HMDB
Chemical FormulaH2O3S
Average Molecular Weight82.079
Monoisotopic Molecular Weight81.97246462
IUPAC Namesulfurous acid
Traditional Namesulfurous acid
CAS Registry Number14265-45-3
SMILES
OS(O)=O
InChI Identifier
InChI=1S/H2O3S/c1-4(2)3/h(H2,1,2,3)
InChI KeyLSNNMFCWUKXFEE-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of inorganic compounds known as non-metal sulfites. These are inorganic non-metallic compounds containing a sulfite as its largest oxoanion.
KingdomInorganic compounds
Super ClassHomogeneous non-metal compounds
ClassNon-metal oxoanionic compounds
Sub ClassNon-metal sulfites
Direct ParentNon-metal sulfites
Alternative Parents
Substituents
  • Non-metal sulfite
  • Inorganic oxide
Molecular FrameworkNot Available
External Descriptors
Ontology
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility558.5 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
logP-1.2ChemAxon
pKa (Strongest Acidic)1.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 ŲChemAxon
Rotatable Bond Count0ChemAxon
Refractivity12.33 m³·mol⁻¹ChemAxon
Polarizability5.76 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-9000000000-6e01fa26fbebd72ad6acView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0ue9-9000000000-f7ae469c0c0eb80e5bb9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-9300000000-8bbaba610a5e9ef617feView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0udi-9300000000-460847a9238bf81b4b9cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-001i-9000000000-0d5af2beca96b50eac8aView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-001i-9000000000-42884a642ace9e213e65View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-01q9-9000000000-5357c61e80aaefe2133cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001i-9000000000-a7737b78e8d12bda4559View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-9000000000-1920e0308a2b8b74a3f4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-001i-9000000000-1920e0308a2b8b74a3f4View in MoNA
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen Locations
  • Blood
  • Urine
Tissue Location
  • Brain
  • Neutrophil
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.23 +/- 0.48 uMAdult (60 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.75 +/- 0.88 uMAdult (60 years old)Both
Acute Infectious Pneumonia
details
UrineDetected and Quantified24 umol/mmol creatinineNewborn (0-30 days old)Not Specified
Molybdenum cofactor deficiency
details
UrineDetected and Quantified66 umol/mmol creatinineChildren (1-13 years old)Not Specified
Molybdenum cofactor deficiency
details
UrineDetected and Quantified500 umol/mmol creatinineInfant (0-1 year old)FemaleMolybdenium co-factor deficiency details
Associated Disorders and Diseases
Disease References
Molybdenum cofactor deficiency
  1. van Gennip AH, Abeling NG, Stroomer AE, Overmars H, Bakker HD: The detection of molybdenum cofactor deficiency: clinical symptomatology and urinary metabolite profile. J Inherit Metab Dis. 1994;17(1):142-5. [PubMed:8051926 ]
Molybdenium co-factor deficiency
  1. Aukett A, Bennett MJ, Hosking GP: Molybdenum co-factor deficiency: an easily missed inborn error of metabolism. Dev Med Child Neurol. 1988 Aug;30(4):531-5. [PubMed:3169394 ]
Associated OMIM IDs
  • 252150 (Molybdenium co-factor deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB013391
KNApSAcK IDC00019662
Chemspider ID1069
KEGG Compound IDC00094
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkSulfurous acid
METLIN IDNot Available
PubChem Compound1100
PDB IDNot Available
ChEBI ID48854
References
Synthesis ReferenceDorain, P. B.; Von Raben, K. U.; Chang, R. K.; Laube, B. L. Catalytic formation of sulfite and sulfate ions from sulfur dioxide on silver observed by surface-enhanced Raman scattering. Chemical Physics Letters (1981), 84(2), 405-9.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Graf WD, Oleinik OE, Jack RM, Weiss AH, Johnson JL: Ahomocysteinemia in molybdenum cofactor deficiency. Neurology. 1998 Sep;51(3):860-2. [PubMed:9748040 ]
  2. Jeppesen C: Media for Aeromonas spp., Plesiomonas shigelloides and Pseudomonas spp. from food and environment. Int J Food Microbiol. 1995 Jun;26(1):25-41. [PubMed:7662518 ]
  3. Mitsuhashi H, Nojima Y, Tanaka T, Ueki K, Maezawa A, Yano S, Naruse T: Sulfite is released by human neutrophils in response to stimulation with lipopolysaccharide. J Leukoc Biol. 1998 Nov;64(5):595-9. [PubMed:9823763 ]
  4. von Graevenitz A, Bucher C: Evaluation of differential and selective media for isolation of Aeromonas and Plesiomonas spp. from human feces. J Clin Microbiol. 1983 Jan;17(1):16-21. [PubMed:6826700 ]
  5. Tsariuk LA, Rybachuk VN, Shevchenko LI, Tolstykh VM: [Determination of fibrinogen concentration in blood plasma by the sulfite precipitation method]. Vopr Med Khim. 1979 Jan-Feb;25(1):97-101. [PubMed:425377 ]
  6. Shea M, Howell S: High-performance liquid chromatographic measurement of exogenous thiosulfate in urine and plasma. Anal Biochem. 1984 Aug 1;140(2):589-94. [PubMed:6486442 ]
  7. Bor-Kucukatay M, Kucukatay V, Agar A, Baskurt OK: Effect of sulfite on red blood cell deformability ex vivo and in normal and sulfite oxidase-deficient rats in vivo. Arch Toxicol. 2005 Sep;79(9):542-6. Epub 2005 Apr 13. [PubMed:15827731 ]
  8. Gubash SM, Ingham L: Comparison of a new, bismuth-iron-sulfite-cycloserine agar for isolation of Clostridium perfringens with the tryptose-sulfite-cycloserine and blood agars. Zentralbl Bakteriol. 1997 Feb;285(3):397-402. [PubMed:9084113 ]
  9. Kim E, Driscoll CF, Minah GE: The effect of a denture adhesive on the colonization of Candida species in vivo. J Prosthodont. 2003 Sep;12(3):187-91. [PubMed:14508740 ]
  10. Willis CL, Cummings JH, Neale G, Gibson GR: Nutritional aspects of dissimilatory sulfate reduction in the human large intestine. Curr Microbiol. 1997 Nov;35(5):294-8. [PubMed:9462959 ]
  11. Sardesai VM: Molybdenum: an essential trace element. Nutr Clin Pract. 1993 Dec;8(6):277-81. [PubMed:8302261 ]
  12. Togawa T, Ogawa M, Nawata M, Ogasawara Y, Kawanabe K, Tanabe S: High performance liquid chromatographic determination of bound sulfide and sulfite and thiosulfate at their low levels in human serum by pre-column fluorescence derivatization with monobromobimane. Chem Pharm Bull (Tokyo). 1992 Nov;40(11):3000-4. [PubMed:1477915 ]
  13. Pearson SJ, Czudek C, Mercer K, Reynolds GP: Electrochemical detection of human brain transmitter amino acids by high-performance liquid chromatography of stable o-phthalaldehyde-sulphite derivatives. J Neural Transm Gen Sect. 1991;86(2):151-7. [PubMed:1683240 ]
  14. Mishra A, Dayal N, Beck-Speier I: Effect of sulphite on the oxidative metabolism of human neutrophils: studies with lucigenin- and luminol-dependent chemiluminescence. J Biolumin Chemilumin. 1995 Jan-Feb;10(1):9-19. [PubMed:7762419 ]
  15. Beck-Speier I, Lenz AG, Godleski JJ: Responses of human neutrophils to sulfite. J Toxicol Environ Health. 1994 Mar;41(3):285-97. [PubMed:8126751 ]
  16. Beck-Speier I, Liese JG, Belohradsky BH, Godleski JJ: Sulfite stimulates NADPH oxidase of human neutrophils to produce active oxygen radicals via protein kinase C and Ca2+/calmodulin pathways. Free Radic Biol Med. 1993 Jun;14(6):661-8. [PubMed:8392022 ]
  17. Zhang X, Vincent AS, Halliwell B, Wong KP: A mechanism of sulfite neurotoxicity: direct inhibition of glutamate dehydrogenase. J Biol Chem. 2004 Oct 8;279(41):43035-45. Epub 2004 Jul 23. [PubMed:15273247 ]
  18. Tan WH, Eichler FS, Hoda S, Lee MS, Baris H, Hanley CA, Grant PE, Krishnamoorthy KS, Shih VE: Isolated sulfite oxidase deficiency: a case report with a novel mutation and review of the literature. Pediatrics. 2005 Sep;116(3):757-66. [PubMed:16140720 ]
  19. Karakas E, Kisker C: Structural analysis of missense mutations causing isolated sulfite oxidase deficiency. Dalton Trans. 2005 Nov 7;(21):3459-63. Epub 2005 Sep 26. [PubMed:16234925 ]
  20. Lester MR: Sulfite sensitivity: significance in human health. J Am Coll Nutr. 1995 Jun;14(3):229-32. [PubMed:8586770 ]
  21. Veldman A, Santamaria-Araujo JA, Sollazzo S, Pitt J, Gianello R, Yaplito-Lee J, Wong F, Ramsden CA, Reiss J, Cook I, Fairweather J, Schwarz G: Successful treatment of molybdenum cofactor deficiency type A with cPMP. Pediatrics. 2010 May;125(5):e1249-54. doi: 10.1542/peds.2009-2192. Epub 2010 Apr 12. [PubMed:20385644 ]

Enzymes

General function:
Involved in thiosulfate sulfurtransferase activity
Specific function:
Transfer of a sulfur ion to cyanide or to other thiol compounds. Also has weak rhodanese activity. May have a role in cyanide degradation or in thiosulfate biosynthesis.
Gene Name:
MPST
Uniprot ID:
P25325
Molecular weight:
33178.15
General function:
Involved in thiosulfate sulfurtransferase activity
Specific function:
Formation of iron-sulfur complexes, cyanide detoxification or modification of sulfur-containing enzymes. Other thiol compounds, besides cyanide, can act as sulfur ion acceptors. Also has weak mercaptopyruvate sulfurtransferase (MST) activity (By similarity). Together with MRPL18, acts as a mitochondrial import factor for the cytosolic 5S rRNA. Only the nascent unfolded cytoplasmic form is able to bind to the 5S rRNA.
Gene Name:
TST
Uniprot ID:
Q16762
Molecular weight:
33428.69
General function:
Involved in heme binding
Specific function:
Not Available
Gene Name:
SUOX
Uniprot ID:
P51687
Molecular weight:
60282.59
General function:
Inorganic ion transport and metabolism
Specific function:
Possible role in tumorgenesis
Gene Name:
TSTD1
Uniprot ID:
Q8NFU3
Molecular weight:
12530.1