创建日期
2005-11-16 15:48:42 UTC
更新日期
2020-04-22 23:32:20 UTC
二级访问
HMDB0001033
HMDB0003008
HMDB00240
HMDB0034829
HMDB01033
HMDB03008
HMDB34829
描述
Endogenous 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.).
同义词
1:[SO(OH)2]
2:Acide sulfureux
3:Acido sulfuroso
4:H2SO3
5:S(O)(OH)2
6:Schweflige saeure
7:Sulphurous acid
8:Sulfurous acid
9:Acide sulphureux
10:Acido sulphuroso
11:Sulphite
12:Sulfur dioxide solution
13:Sulfuric(IV) acid (H2SO3)
14:Hydrogen sulfite
15:Sulfite
inchi标识符
InChI=1S/H2O3S/c1-4(2)3/h(H2,1,2,3)
印记键
LSNNMFCWUKXFEE-UHFFFAOYSA-N
分类学
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.
description:Non-metal sulfites
kingdom:Inorganic compounds
super_class:Homogeneous non-metal compounds
class:Non-metal oxoanionic compounds
sub_class:Non-metal sulfites
molecular_framework:
1:Inorganic oxides
2:Inorganic oxide
3:Non-metal sulfite
4:a small molecule
5:sulfur oxoacid
本体论
term:Disposition
definition:A concept that describes the origin of a chemical, its location within an organism, or its route of exposure.
parent_id:
level:1
type:parent
term:Route of exposure
definition:A mean by which a chemical agent comes in contact with an organism, either under intended or unintended circumstances.
parent_id:7724
*level:2
type:parent
term:Enteral
definition:Chemical exposure via the alimentary canal (mouth to anus).
parent_id:7743
**level:3
type:parent
term:Ingestion
definition:Chemical exposure facilitated by entry through the mouth.
parent_id:7744
***level:4
type:child
synonym:Digestion
term:Source
definition:Natural or synthetic origin of a chemical.
parent_id:7724
*level:2
type:parent
term:Endogenous
definition:
parent_id:7735
**level:3
type:child
term:Food
definition:
parent_id:7735
**level:3
type:child
term:Biological
definition:A living organism (species or a higher taxonomy rank), in which a chemical can be found.
parent_id:7735
**level:3
type:parent
term:Animal
definition:A living organism belonging to the kingdom animalia. it feeds on organic matter, typically having specialized sense organs and nervous system and able to respond rapidly to stimuli.
parent_id:7736
***level:4
type:child
synonym:Fauna
term:Plant
definition:A living organism belonging to the kingdom plantea. typically, it grows in a permanent site, absorbs water and inorganic substances through its roots, and synthesizes nutrients in its leaves by photosynthesis using the green pigment chlorophyll. examples incude trees, shrubs, herbs, grasses, ferns, and mosses.
parent_id:7736
***level:4
type:parent
synonym:Flora
term:Biological location
definition:The physiological origin within an organism, including anatomical compnents, biofluids and excreta.
parent_id:7724
*level:2
type:parent
term:Organ and components
definition:An anatomical organizational level including multiple tissues or substructures, which enables a common biological function.
parent_id:7725
**level:3
type:parent
term:Brain
definition:An organ composed of grey and white matter that is the center for intelligence and reasoning. it is protected by the bony cranium. (nci).
parent_id:7727
***level:4
type:child
term:Biofluid and excreta
definition:A liquid, semi-solid or solid material originating in the body.
parent_id:7725
**level:3
type:parent
term:Blood
definition:A liquid tissue with the primary function of transporting oxygen and carbon dioxide (nci). it supplies the tissues with nutrients, removes waste products, and contains various components of the immune system defending the body against infection.
parent_id:7731
***level:4
type:child
term:Subcellular
definition:An anatomical organizational level including a component within a biological cell .
parent_id:7725
**level:3
type:parent
term:Mitochondria
definition:
parent_id:7730
***level:4
type:child
term:Cytoplasm
definition:The portion of the cell contained within the plasma membrane but excluding the nucleus.
parent_id:7730
***level:4
type:child
synonym:Cytoplasma
term:Process
definition:Biological or chemical events, or a series thereof, leading to a known function or end-product.
parent_id:
level:1
type:parent
term:Naturally occurring process
definition:Naturally-occurring molecular events or a series thereof, leading to a known function or end-product.
parent_id:7659
*level:2
type:parent
term:Biological process
definition:Biological or chemical events or a series thereof, leading to a known function or end-product within an organism.
parent_id:7660
**level:3
type:parent
term:Biochemical pathway
definition:A linked series of chemical reactions that occur in a defined order within or between organism cells, and lead to a known function or end product.
parent_id:7661
***level:4
type:parent
term:Role
definition:The purpose or function assumed by a chemical, either naturally or as intended by humans .
parent_id:
level:1
type:parent
term:Indirect biological role
definition:An indirect function of a chemical which affect the physiological state of any organism (apart from the source organism, if of biological source).
parent_id:7671
*level:2
type:parent
term:Allergen
definition:A chemical substance or preparation that causes an allergic reaction in the body of animals and humans.
parent_id:7691
**level:3
type:child
synonym:Allergic agent
term:Neurotoxin
definition:A chemical substance that interferes with the normal functioning of the nervous system.
parent_id:7691
**level:3
type:child
synonym:Neurotoxic
synonym:Neurotoxic agent
term:Metabotoxin
definition:An endogenous metabolite that causes adverse health effects at chronically high levels.
parent_id:7691
**level:3
type:child
term:Biological role
definition:The biological function of a chemical. the biological role answers the question how a chemical is involved in molecular processes in an organism. this can include biochemical effects of non-endogenous chemicals, which are also assigned an industrial application such as pharmaceuticals. the biological role is limited to cellular levls, and will not include role at system process level, such as a chemical which has a role in a disease.
parent_id:7671
*level:2
type:parent
term:Osmolyte
definition:A chemical substance that affects osmosis. osmolytes are soluble in the cell or in the fluid between cells, and are utilized by cells during water stress to maintain the cell volume.
parent_id:7672
**level:3
type:child
synonym:Osmolosis agent
term:Nutrient
definition:A chamical substance in food, further ingested and utilized by an organism for its well-being, growth and reproduction.
parent_id:7672
**level:3
type:child
term:Molecular messenger
definition: a chemical substance that is involved in transmission of biochemical signals.
parent_id:7672
**level:3
type:parent
term:Signaling molecule
definition:A chemical substance that particpiates in signal transduction processes in a living cell.
parent_id:7677
***level:4
type:child
synonym:Signalling molecule
synonym:Signaling agent
synonym:Cell signaling agent
实验性质
1:
kind:water_solubility
value:558.5 mg/mL
source:
预测性质
1:
kind:logp
value:-1.2
source:ChemAxon
2:
kind:pka_strongest_acidic
value:1.7
source:ChemAxon
3:
kind:iupac
value:sulfurous acid
source:ChemAxon
4:
kind:average_mass
value:82.079
source:ChemAxon
5:
kind:mono_mass
value:81.97246462
source:ChemAxon
6:
kind:smiles
value:OS(O)=O
source:ChemAxon
7:
kind:formula
value:H2O3S
source:ChemAxon
8:
kind:inchi
value:InChI=1S/H2O3S/c1-4(2)3/h(H2,1,2,3)
source:ChemAxon
9:
kind:inchikey
value:LSNNMFCWUKXFEE-UHFFFAOYSA-N
source:ChemAxon
10:
kind:polar_surface_area
value:57.53
source:ChemAxon
11:
kind:refractivity
value:12.33
source:ChemAxon
12:
kind:polarizability
value:5.76
source:ChemAxon
13:
kind:rotatable_bond_count
value:0
source:ChemAxon
14:
kind:acceptor_count
value:3
source:ChemAxon
15:
kind:donor_count
value:2
source:ChemAxon
16:
kind:physiological_charge
value:-1
source:ChemAxon
17:
kind:formal_charge
value:0
source:ChemAxon
18:
kind:number_of_rings
value:0
source:ChemAxon
19:
kind:bioavailability
value:Yes
source:ChemAxon
20:
kind:rule_of_five
value:Yes
source:ChemAxon
21:
kind:ghose_filter
value:No
source:ChemAxon
22:
kind:veber_rule
value:No
source:ChemAxon
23:
kind:mddr_like_rule
value:No
source:ChemAxon
生物学性质
1:
cellular:(1):Mitochondria
2:
biospecimen:(1):Blood
biospecimen:(2):Urine
3:
tissue:(1):Brain
tissue:(2):Leukocyte
4:
[1]:
name:Beta-mercaptolactate-cysteine disulfiduria
smpdb_id:SMP00499
kegg_map_id:
[2]:
name:Cysteine Metabolism
smpdb_id:SMP00013
kegg_map_id:map00270
[3]:
name:Cystinosis, ocular nonnephropathic
smpdb_id:SMP00722
kegg_map_id:
[4]:
name:Sulfite oxidase deficiency
smpdb_id:SMP00532
kegg_map_id:
[5]:
name:Sulfur metabolism
smpdb_id:
kegg_map_id:map00920
正常浓度
1:
biospecimen:Blood
concentration_value:1.23 +/- 0.48
concentration_units:uM
subject_age:Adult (60 years old)
subject_sex:Both
subject_condition:Normal
异常浓度
1:
biospecimen:Blood
concentration_value:3.75 +/- 0.88
concentration_units:uM
patient_age:Adult (60 years old)
patient_sex:Both
patient_information:Acute Infectious Pneumonia
2:
biospecimen:Urine
concentration_value:24
concentration_units:umol/mmol creatinine
patient_age:Newborn (0-30 days old)
patient_sex:Not Specified
patient_information:Molybdenum cofactor deficiency
3:
biospecimen:Urine
concentration_value:66
concentration_units:umol/mmol creatinine
patient_age:Children (1-13 years old)
patient_sex:Not Specified
patient_information:Molybdenum cofactor deficiency
4:
biospecimen:Urine
concentration_value:500
concentration_units:umol/mmol creatinine
patient_age:Infant (0-1 year old)
patient_sex:Female
patient_information:Molybdenium co-factor deficiency
疾病参考
1:
name:Molybdenum cofactor deficiency
omim_id:
[1]:
reference_text:Wevers RA, Engelke UF, Moolenaar SH, Brautigam C, de Jong JG, Duran R, de Abreu RA, van Gennip AH: 1H-NMR spectroscopy of body fluids: inborn errors of purine and pyrimidine metabolism. Clin Chem. 1999 Apr;45(4):539-48.
pubmed_id:10102915
[2]:
reference_text: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_id:8051926
[3]:
reference_text:Zaki MS, Selim L, El-Bassyouni HT, Issa MY, Mahmoud I, Ismail S, Girgis M, Sadek AA, Gleeson JG, Abdel Hamid MS: Molybdenum cofactor and isolated sulphite oxidase deficiencies: Clinical and molecular spectrum among Egyptian patients. Eur J Paediatr Neurol. 2016 Sep;20(5):714-22. doi: 10.1016/j.ejpn.2016.05.011. Epub 2016 May 30.
pubmed_id:27289259
[4]:
reference_text:Sass JO, Kishikawa M, Puttinger R, Reiss J, Erwa W, Shimizu A, Sperl W: Hypohomocysteinaemia and highly increased proportion of S-sulfonated plasma transthyretin in molybdenum cofactor deficiency. J Inherit Metab Dis. 2003;26(1):80-2.
pubmed_id:12872846
[5]:
reference_text:MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
pubmed_id:
2:
name:Molybdenium co-factor deficiency
omim_id:252150
[1]:
reference_text: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_id:3169394
[2]:
reference_text:Nagappa M, Bindu PS, Taly AB, Sinha S, Bharath RD: Child Neurology: Molybdenum cofactor deficiency. Neurology. 2015 Dec 8;85(23):e175-8. doi: 10.1212/WNL.0000000000002194.
pubmed_id:26644055
综合参考
Dorain, 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.
一般参考
1:
protein_accession:HMDBP00781
name:3-mercaptopyruvate sulfurtransferase
uniprot_id:P25325
gene_name:MPST
protein_type:Enzyme
2:
protein_accession:HMDBP01056
name:Thiosulfate sulfurtransferase
uniprot_id:Q16762
gene_name:TST
protein_type:Unknown
3:
protein_accession:HMDBP01057
name:Sulfite oxidase, mitochondrial
uniprot_id:P51687
gene_name:SUOX
protein_type:Unknown
4:
protein_accession:HMDBP09168
name:Thiosulfate sulfurtransferase/rhodanese-like domain-containing protein 1
uniprot_id:Q8NFU3
gene_name:TSTD1
protein_type:Enzyme
蛋白质结合
1:
reference_text:Graf WD, Oleinik OE, Jack RM, Weiss AH, Johnson JL: Ahomocysteinemia in molybdenum cofactor deficiency. Neurology. 1998 Sep;51(3):860-2.
pubmed_id:9748040
2:
reference_text: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_id:7662518
3:
reference_text: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_id:9823763
4:
reference_text: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_id:6826700
5:
reference_text: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_id:425377
6:
reference_text: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_id:6486442
7:
reference_text: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_id:15827731
8:
reference_text: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_id:9084113
9:
reference_text: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_id:14508740
10:
reference_text: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_id:9462959
11:
reference_text:Sardesai VM: Molybdenum: an essential trace element. Nutr Clin Pract. 1993 Dec;8(6):277-81.
pubmed_id:8302261
12:
reference_text: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_id:1477915
13:
reference_text: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_id:1683240
14:
reference_text: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_id:7762419
15:
reference_text:Beck-Speier I, Lenz AG, Godleski JJ: Responses of human neutrophils to sulfite. J Toxicol Environ Health. 1994 Mar;41(3):285-97.
pubmed_id:8126751
16:
reference_text: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_id:8392022
17:
reference_text: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_id:15273247
18:
reference_text: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_id:16140720
19:
reference_text: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_id:16234925
20:
reference_text:Lester MR: Sulfite sensitivity: significance in human health. J Am Coll Nutr. 1995 Jun;14(3):229-32.
pubmed_id:8586770
21:
reference_text: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_id:20385644
22:
reference_text:Yannai, Shmuel. (2004) Dictionary of food compounds with CD-ROM: Additives, flavors, and ingredients. Boca Raton: Chapman & Hall/CRC.