Page last updated: 2024-10-06

Sulfur amino acid metabolism

Proteins (27)

ProteinSynonymsTaxonomy
Aspartate aminotransferase, mitochondrialmAspAT; EC 2.6.1.1; EC 2.6.1.7; Fatty acid-binding protein; FABP-1; Glutamate oxaloacetate transaminase 2; Kynurenine aminotransferase 4; Kynurenine aminotransferase IV; Kynurenine--oxoglutarate transaminase 4; Kynurenine--oxoglutarate transaminase IV; PlHomo sapiens (human)
Cystathionine gamma-lyaseEC 4.4.1.1; Cysteine-protein sulfhydrase; Gamma-cystathionaseHomo sapiens (human)
Sulfide:quinone oxidoreductase, mitochondrialSQOR; EC 1.8.5.8; Sulfide dehydrogenase-like; Sulfide quinone oxidoreductaseHomo sapiens (human)
AdenosylhomocysteinaseAdoHcyase; EC 3.3.1.1; S-adenosyl-L-homocysteine hydrolaseHomo sapiens (human)
Dimethylaniline monooxygenase [N-oxide-forming] 1EC 1.14.13.8; Dimethylaniline oxidase 1; Fetal hepatic flavin-containing monooxygenase 1; FMO 1Homo sapiens (human)
S-methylmethionine--homocysteine S-methyltransferase BHMT2SMM-hcy methyltransferase; EC 2.1.1.10; Betaine--homocysteine S-methyltransferase 2Homo sapiens (human)
Aspartate aminotransferase, cytoplasmiccAspAT; EC 2.6.1.1; EC 2.6.1.3; Cysteine aminotransferase, cytoplasmic; Cysteine transaminase, cytoplasmic; cCAT; Glutamate oxaloacetate transaminase 1; Transaminase AHomo sapiens (human)
S-methyl-5'-thioadenosine phosphorylaseEC 2.4.2.28; 5'-methylthioadenosine phosphorylase; MTA phosphorylase; MTAP; MTAPaseHomo sapiens (human)
Betaine--homocysteine S-methyltransferase 1EC 2.1.1.5Homo sapiens (human)
Thioredoxin, mitochondrialMTRX; Mt-Trx; Thioredoxin-2Homo sapiens (human)
Cystathionine beta-synthaseEC 4.2.1.22; Beta-thionase; Serine sulfhydraseHomo sapiens (human)
Methionine synthaseMS; EC 2.1.1.13; 5-methyltetrahydrofolate--homocysteine methyltransferase; Cobalamin-dependent methionine synthase; Vitamin-B12 dependent methionine synthaseHomo sapiens (human)
Thiosulfate:glutathione sulfurtransferaseTST; EC 2.8.1.-Homo sapiens (human)
Cysteine dioxygenase type 1EC 1.13.11.20; Cysteine dioxygenase type I; CDO; CDO-IHomo sapiens (human)
Cysteine sulfinic acid decarboxylaseEC 4.1.1.29; Aspartate 1-decarboxylase; 4.1.1.11; Cysteine-sulfinate decarboxylase; Sulfinoalanine decarboxylaseHomo sapiens (human)
Thiosulfate sulfurtransferaseEC 2.8.1.1; RhodaneseHomo sapiens (human)
Persulfide dioxygenase ETHE1, mitochondrialEC 1.13.11.18; Ethylmalonic encephalopathy protein 1; Hepatoma subtracted clone one protein; Sulfur dioxygenase ETHE1Homo sapiens (human)
1,2-dihydroxy-3-keto-5-methylthiopentene dioxygenaseEC 1.13.11.54; Acireductone dioxygenase (Fe(2+)-requiring); ARD; Fe-ARD; Membrane-type 1 matrix metalloproteinase cytoplasmic tail-binding protein 1; MTCBP-1; Submergence-induced protein-like factor; Sip-LHomo sapiens (human)
Sulfite oxidase, mitochondrialEC 1.8.3.1Homo sapiens (human)
Methionine synthase reductase MSR; EC 1.16.1.8; Aquacobalamin reductase; AqCbl reductase; 1.16.1.-Homo sapiens (human)
2-aminoethanethiol dioxygenaseEC 1.13.11.19; Cysteamine dioxygenaseHomo sapiens (human)
Acidic amino acid decarboxylase GADL1Aspartate 1-decarboxylase; ADC; HuADC; 4.1.1.11; Cysteine sulfinic acid decarboxylase; CSADC; HuCSADC; 4.1.1.29; Glutamate decarboxylase-like protein 1Homo sapiens (human)
3-mercaptopyruvate sulfurtransferaseMST; EC 2.8.1.2Homo sapiens (human)
Enolase-phosphatase E1EC 3.1.3.77; 2,3-diketo-5-methylthio-1-phosphopentane phosphatase; MASA homologHomo sapiens (human)
Methylthioribose-1-phosphate isomeraseM1Pi; MTR-1-P isomerase; EC 5.3.1.23; Mediator of RhoA-dependent invasion; S-methyl-5-thioribose-1-phosphate isomerase; Translation initiation factor eIF-2B subunit alpha/beta/delta-like proteinHomo sapiens (human)
Methylthioribulose-1-phosphate dehydrataseMTRu-1-P dehydratase; EC 4.2.1.109; APAF1-interacting protein; hAPIPHomo sapiens (human)
Mitochondrial dicarboxylate carrierSolute carrier family 25 member 10Homo sapiens (human)

Compounds (46)

CompoundDescription
hydronium ion
hydrogen sulfite
GlutathioneA tripeptide with many roles in cells. It conjugates to drugs to make them more soluble for excretion, is a cofactor for some enzymes, is involved in protein disulfide bond rearrangement and reduces peroxides.
adenineA purine base and a fundamental unit of ADENINE NUCLEOTIDES.
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
betaineA naturally occurring compound that has been of interest for its role in osmoregulation. As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1341)
ironA metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
ZincA metallic element of atomic number 30 and atomic weight 65.38. It is a necessary trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Zinc deficiency is associated with ANEMIA, short stature, HYPOGONADISM, impaired WOUND HEALING, and geophagia. It is known by the symbol Zn.
Orthophosphate
Glutathione DisulfideA GLUTATHIONE dimer formed by a disulfide bond between the cysteine sulfhydryl side chains during the course of being oxidized.
Flavin MononucleotideA coenzyme for a number of oxidative enzymes including NADH DEHYDROGENASE. It is the principal form in which RIBOFLAVIN is found in cells and tissues.
ubiquinol 1
coenzyme q10Ubiquinone ring with a chain of 10 isoprene units; redox equilibrium with ubiqunol serving in mitochondrial inner membrane to transfer electrons; presence during reconstitution of acetylcholine receptor into phospholipid vesicles yields vesicles active in catalyzing carbamylcholine-sensitive Na+ flux; coenzyme Q10 depletion has been noted with use of statins
Cobalamins
adenosineA nucleoside that is composed of ADENINE and D-RIBOSE. Adenosine or adenosine derivatives play many important biological roles in addition to being components of DNA and RNA. Adenosine itself is a neurotransmitter.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
MagnesiumA metallic element that has the atomic symbol Mg, atomic number 12, and atomic weight 24.31. It is important for the activity of many enzymes, especially those involved in OXIDATIVE PHOSPHORYLATION.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
5'-methylthioribose 1-phosphate
Pyruvic AcidAn intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed)
sulfitesInorganic salts of sulfurous acid.
Methylmethionine Sulfonium Chloride
nadA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
NADPNicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
NADPNicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)
alpha-Ketoglutarate
formate
5'-methylthioadenosinestructure
CystathionineSulfur-containing amino acid formed as an intermediate in the conversion of METHIONINE to CYSTEINE.
2-keto-4-thiomethylbutyrate
glutamate
adenosine triphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
alpha-ketobutyric acidRN given refers to parent cpd; structure
CysteineA thiol-containing non-essential amino acid that is oxidized to form CYSTINE.
hydrogen cyanideHydrogen cyanide (HCN); A toxic liquid or colorless gas. It is found in the smoke of various tobacco products and released by combustion of nitrogen-containing organic materials.
3-mercaptopyruvic acidRN given refers to parent cpd; structure
cysteine sulfinate
1-phospho-5-s-methylthioribulose
s-adenosylmethioninePhysiologic methyl radical donor involved in enzymatic transmethylation reactions and present in all living organisms. It possesses anti-inflammatory activity and has been used in treatment of chronic liver disease. (From Merck, 11th ed)
2,5-dihydroxy-1,4-dithiane-2,5-dicarboxylic acidprodrug of 3-mercaptopyruvate; antidote to cyanide poisoning; structure in first source
Flavin-Adenine DinucleotideA condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972)
hemeThe color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.
thiocyanateRN given refers to parent cpd
lanthioninea thioether analogue of cystine; RN given refers to (DL)-isomer; structure; a component of lantibiotics (BACTERIOCINS)