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Methionine metabolism leading to sulfur amino acids and related disorders

Proteins (9)

ProteinSynonymsTaxonomy
S-adenosylmethionine synthase isoform type-1AdoMet synthase 1; EC 2.5.1.6; Methionine adenosyltransferase 1; MAT 1; Methionine adenosyltransferase I/III; MAT-I/IIIHomo sapiens (human)
AdenosylhomocysteinaseAdoHcyase; EC 3.3.1.1; S-adenosyl-L-homocysteine hydrolaseHomo sapiens (human)
Glycine N-methyltransferaseEC 2.1.1.20Homo sapiens (human)
Methionine synthaseMS; EC 2.1.1.13; 5-methyltetrahydrofolate--homocysteine methyltransferase; Cobalamin-dependent methionine synthase; Vitamin-B12 dependent methionine synthaseHomo 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)
S-adenosylmethionine synthase Homo sapiens (human)
Betaine--homocysteine S-methyltransferase 1Homo sapiens (human)
Methionine adenosyltransferase 2 subunit beta Homo sapiens (human)

Compounds (17)

CompoundDescription
sulfuric acid
taurineA conditionally essential nutrient, important during mammalian development. It is present in milk but is isolated mostly from ox bile and strongly conjugates bile acids.
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)
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)
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.
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
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.
methionineA sulfur-containing essential L-amino acid that is important in many body functions.
glycineA non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter.
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)
sarcosineAn amino acid intermediate in the metabolism of choline.
s-sulphocysteineresidues in physiologically significant proteins treated with sulfite; RN given refers to parent cpd; structure
CystathionineSulfur-containing amino acid formed as an intermediate in the conversion of METHIONINE to CYSTEINE.
HomocysteineA thiol-containing amino acid formed by a demethylation of METHIONINE.
hypotaurineintermediate in production of taurine in the liver; structure
cysteine sulfinate
s-adenosylhomocysteine5'-S-(3-Amino-3-carboxypropyl)-5'-thioadenosine. Formed from S-adenosylmethionine after transmethylation reactions.