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Showing metabocard for Sulfite (HMDB0000240)

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enzymes (4) Show 4 proteins
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:38 UTC
HMDB IDHMDB0000240
Secondary Accession Numbers
  • HMDB0001033
  • HMDB0003008
  • HMDB00240
  • HMDB0034829
  • HMDB01033
  • HMDB03008
  • HMDB34829
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 ). The hydrogen sulfite, or bisulfite, ion is the ion HSO3-. It is the conjugate base of sulfurous acid, H2SO3. Bisulfite has long been recognized as a reagent to react with organic compounds in various ways; prominent among them are additions to carbonyl groups and to carbon-carbon double bonds, and free radical reactions in the presence of oxygen. Bisulfite reacts with pyrimidine nucleosides, undergoing additions to the 5,6-double bond to form pyrimidine-5,6-dihydro-6-sulfonates. The addition across the 5,6-double bond is reversible. All these adducts are unstable in alkali. Bisulfite modification has been used to probe secondary or higher structures of polynucleotides as it reacts with pyrimidines in single-stranded regions specifically. In animal DNA, a portion of the pyrimidine base cytosine is methylated at position 5. 5-Methylcytosine in DNA is now an intensive focus of attention for its roles in gene functions. The methylation occurs by postreplication modification and is a heritable event. 5-Methylcytosine sites are known to be mutation hot spots. 5-Methylcytosine spontaneously deaminates into thymine, while cytosine does so more slowly. Determination of the position of 5-methylcytosine in a given DNA requires some means to distinguish 5-methylcytosine from cytosine. Chemical modification can be used as one such means. Treatment of DNA with bisulfite converts cytosine into uracil by deamination, while 5-methylcytosine remains mostly unaltered. The majority of recent research on 5-methylcytosine in DNA employs bisulfite treatment in the analytical procedure. The principle of this procedure is as follows. As uracil is a thymine-analog (5-methyluracil is thymine), it behaves toward DNA polymerases as thymine. When the bisulfite-modified DNA is subjected to PCR (polymerase chain reaction), a process necessary to amplify tiny samples of DNA, the uracil residues will become thymine residues in the amplified products. As 5-methylcytosine residues in the original DNA sample remain unaltered during the bisulfite treatment, the amplification will produce polynucleotides in which cytosine residues represent the 5-methylcytosine residues of the original (Genes and Environment (2006), 28(1), 1-8.).
Structure
Data?1676999678
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000240 (Sulfite)


  Mrv1652305171818402D          

  4  3  0  0  0  0            999 V2000
    1.2375    0.7145    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.8250    0.0000    0.0000 S   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375   -0.7145    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    0.0000    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  2  0  0  0  0
  4  2  1  0  0  0  0
M  END
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3D MOL for HMDB0000240 (Sulfite)

HMDB0000240
     RDKit          3D
Sulfite
  6  5  0  0  0  0  0  0  0  0999 V2000
    0.0395    1.8868   -0.3873 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0597    0.6001    0.3857 S   0  0  0  0  0  4  0  0  0  0  0  0
   -1.4799   -0.2436    0.1609 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3230   -0.3040    0.4520 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3897   -1.1282   -0.2634 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.5668   -0.8111   -0.3479 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  3  5  1  0
  4  6  1  0
M  END
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3D SDF for HMDB0000240 (Sulfite)


  Mrv1652305171818402D          

  4  3  0  0  0  0            999 V2000
    1.2375    0.7145    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.8250    0.0000    0.0000 S   0  0  0  0  0  0  0  0  0  0  0  0
    1.2375   -0.7145    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0000    0.0000    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  2  0  0  0  0
  4  2  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000240

> <DATABASE_NAME>
hmdb

> <SMILES>
OS(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/H2O3S/c1-4(2)3/h(H2,1,2,3)

> <INCHI_KEY>
LSNNMFCWUKXFEE-UHFFFAOYSA-N

> <FORMULA>
H2O3S

> <MOLECULAR_WEIGHT>
82.079

> <EXACT_MASS>
81.97246462

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
6

> <JCHEM_AVERAGE_POLARIZABILITY>
5.756159735620625

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
sulfurous acid

> <JCHEM_LOGP>
-1.1546872626666664

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
7.30102999566398

> <JCHEM_PKA_STRONGEST_ACIDIC>
1.6989700043360187

> <JCHEM_POLAR_SURFACE_AREA>
57.53

> <JCHEM_REFRACTIVITY>
12.3279

> <JCHEM_ROTATABLE_BOND_COUNT>
0

> <JCHEM_RULE_OF_FIVE>
1

> <JCHEM_TRADITIONAL_IUPAC>
sulfurous acid

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for HMDB0000240 (Sulfite)

HMDB0000240
     RDKit          3D
Sulfite
  6  5  0  0  0  0  0  0  0  0999 V2000
    0.0395    1.8868   -0.3873 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0597    0.6001    0.3857 S   0  0  0  0  0  4  0  0  0  0  0  0
   -1.4799   -0.2436    0.1609 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3230   -0.3040    0.4520 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3897   -1.1282   -0.2634 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.5668   -0.8111   -0.3479 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  3  5  1  0
  4  6  1  0
M  END
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PDB for HMDB0000240 (Sulfite)

HEADER    PROTEIN                                 17-MAY-18   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   17-MAY-18         0                                  
HETATM    1  O   UNK     0       2.310   1.334   0.000  0.00  0.00           O+0
HETATM    2  S   UNK     0       1.540   0.000   0.000  0.00  0.00           S+0
HETATM    3  O   UNK     0       2.310  -1.334   0.000  0.00  0.00           O+0
HETATM    4  O   UNK     0       0.000   0.000   0.000  0.00  0.00           O+0
CONECT    1    2                                                            
CONECT    2    1    3    4                                                  
CONECT    3    2                                                            
CONECT    4    2                                                            
MASTER        0    0    0    0    0    0    0    0    4    0    6    0
END
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3D PDB for HMDB0000240 (Sulfite)

COMPND    HMDB0000240
HETATM    1  O1  UNL     1       0.040   1.887  -0.387  1.00  0.00           O  
HETATM    2  S1  UNL     1      -0.060   0.600   0.386  1.00  0.00           S  
HETATM    3  O2  UNL     1      -1.480  -0.244   0.161  1.00  0.00           O  
HETATM    4  O3  UNL     1       1.323  -0.304   0.452  1.00  0.00           O  
HETATM    5  H1  UNL     1      -1.390  -1.128  -0.263  1.00  0.00           H  
HETATM    6  H2  UNL     1       1.567  -0.811  -0.348  1.00  0.00           H  
CONECT    1    2    2
CONECT    2    3    4
CONECT    3    5
CONECT    4    6
END
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SMILES for HMDB0000240 (Sulfite)

OS(O)=O
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INCHI for HMDB0000240 (Sulfite)

InChI=1S/H2O3S/c1-4(2)3/h(H2,1,2,3)
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View in JSmolView Stereo Labels
Synonyms
ValueSource
[SO(OH)2]ChEBI
Acide sulfureuxChEBI
Acido sulfurosoChEBI
H2SO3ChEBI
S(O)(OH)2ChEBI
Schweflige saeureChEBI
Sulphurous acidChEBI
Sulfurous acidKegg
Acide sulphureuxGenerator
Acido sulphurosoGenerator
SulphiteGenerator
Sulfur dioxide solutionHMDB
Sulfuric(IV) acid (H2SO3)HMDB
Hydrogen sulfiteHMDB
SulfiteChEBI
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
Description 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
Physiological effectNot Available
Disposition
Source
ProcessNot Available
RoleNot Available
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility558.5 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular 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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+111.79231661259
DarkChem[M+H]+111.79231661259
DarkChem[M+H]+111.79231661259
DarkChem[M+H]+111.79231661259
DarkChem[M+H]+111.79231661259
DarkChem[M+H]+111.79231661259
DarkChem[M-H]-102.96331661259
DarkChem[M-H]-102.96331661259
DarkChem[M-H]-102.96331661259
DarkChem[M-H]-102.96331661259
DarkChem[M-H]-102.96331661259
DarkChem[M-H]-102.96331661259
AllCCS[M+H]+112.20232859911
AllCCS[M-H]-144.62332859911
DeepCCS[M+H]+126.70430932474
DeepCCS[M-H]-124.57330932474
DeepCCS[M-2H]-160.71730932474
DeepCCS[M+Na]+135.12330932474
AllCCS[M+H]+112.232859911
AllCCS[M+H-H2O]+107.832859911
AllCCS[M+NH4]+116.332859911
AllCCS[M+Na]+117.532859911
AllCCS[M-H]-144.632859911
AllCCS[M+Na-2H]-151.532859911
AllCCS[M+HCOO]-159.032859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
SulfiteOS(O)=O1852.3Standard polar33892256
SulfiteOS(O)=O783.9Standard non polar33892256
SulfiteOS(O)=O854.3Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Sulfite,1TMS,isomer #1C[Si](C)(C)OS(=O)O1009.7Semi standard non polar33892256
Sulfite,1TMS,isomer #1C[Si](C)(C)OS(=O)O1015.5Standard non polar33892256
Sulfite,1TMS,isomer #1C[Si](C)(C)OS(=O)O1425.4Standard polar33892256
Sulfite,2TMS,isomer #1C[Si](C)(C)OS(=O)O[Si](C)(C)C1062.3Semi standard non polar33892256
Sulfite,2TMS,isomer #1C[Si](C)(C)OS(=O)O[Si](C)(C)C1190.7Standard non polar33892256
Sulfite,2TMS,isomer #1C[Si](C)(C)OS(=O)O[Si](C)(C)C1083.3Standard polar33892256
Sulfite,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OS(=O)O1259.7Semi standard non polar33892256
Sulfite,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OS(=O)O1304.5Standard non polar33892256
Sulfite,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OS(=O)O1542.4Standard polar33892256
Sulfite,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OS(=O)O[Si](C)(C)C(C)(C)C1491.4Semi standard non polar33892256
Sulfite,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OS(=O)O[Si](C)(C)C(C)(C)C1704.4Standard non polar33892256
Sulfite,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OS(=O)O[Si](C)(C)C(C)(C)C1398.6Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - Sulfite GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-9000000000-6e01fa26fbebd72ad6ac2017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Sulfite GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Sulfite Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-0ue9-9000000000-f7ae469c0c0eb80e5bb92018-05-15HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Sulfite Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-001i-9300000000-8bbaba610a5e9ef617fe2018-05-15HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Sulfite Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0udi-9300000000-460847a9238bf81b4b9c2018-05-15HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 10V, Positive-QTOFsplash10-001i-9000000000-0d5af2beca96b50eac8a2015-09-14Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 20V, Positive-QTOFsplash10-001i-9000000000-42884a642ace9e213e652015-09-14Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 40V, Positive-QTOFsplash10-01q9-9000000000-5357c61e80aaefe2133c2015-09-14Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 10V, Negative-QTOFsplash10-001i-9000000000-a7737b78e8d12bda45592015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 20V, Negative-QTOFsplash10-001i-9000000000-1920e0308a2b8b74a3f42015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 40V, Negative-QTOFsplash10-001i-9000000000-1920e0308a2b8b74a3f42015-09-15Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 10V, Negative-QTOFsplash10-001i-9000000000-a6fb8cd4d3dc149be3092021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 20V, Negative-QTOFsplash10-001i-9000000000-a6fb8cd4d3dc149be3092021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 40V, Negative-QTOFsplash10-001i-9000000000-a6fb8cd4d3dc149be3092021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 10V, Positive-QTOFsplash10-001i-9000000000-95d8eaf2b829c52ced6c2021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 20V, Positive-QTOFsplash10-03di-9000000000-2c4a3db8921d94d7f5262021-09-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Sulfite 40V, Positive-QTOFsplash10-03di-9000000000-2c4a3db8921d94d7f5262021-09-24Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen Locations
  • Blood
  • Urine
Tissue Locations
  • Brain
  • Leukocyte
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
FooDB IDFDB013391
KNApSAcK IDC00019662
Chemspider ID1069
KEGG Compound IDC00094
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkSulfite
METLIN IDNot Available
PubChem Compound1100
PDB IDNot Available
ChEBI ID48854
Food Biomarker OntologyNot Available
VMH IDSO3
MarkerDB IDNot Available
Good Scents IDNot Available
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 ]
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Enzymes

Enzyme Details
1. 3-mercaptopyruvate sulfurtransferase
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
Reactions
Thiosulfate + Hydrogen cyanide → Sulfite + Thiocyanatedetails
3-Mercaptopyruvic acid + Sulfite → Thiosulfate + Pyruvic aciddetails
Enzyme Details
2. Thiosulfate sulfurtransferase
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
Reactions
Thiosulfate + Hydrogen cyanide → Sulfite + Thiocyanatedetails
3-Mercaptopyruvic acid + Sulfite → Thiosulfate + Pyruvic aciddetails
Enzyme Details
3. Sulfite oxidase, mitochondrial
General function:
Involved in heme binding
Specific function:
Not Available
Gene Name:
SUOX
Uniprot ID:
P51687
Molecular weight:
60282.59
Reactions
Sulfite + Oxygen + Water → Oat gum + Hydrogen peroxidedetails
Enzyme Details
4. Thiosulfate sulfurtransferase/rhodanese-like domain-containing protein 1
General function:
Inorganic ion transport and metabolism
Specific function:
Possible role in tumorgenesis
Gene Name:
TSTD1
Uniprot ID:
Q8NFU3
Molecular weight:
12530.1