Page last updated: 2024-10-06

S-Adenosylhomocysteine (SAH) Hydrolase Deficiency

Proteins (16)

ProteinSynonymsTaxonomy
Cystathionine gamma-lyaseEC 4.4.1.1; Cysteine-protein sulfhydrase; Gamma-cystathionaseHomo sapiens (human)
Methionine--tRNA ligase, cytoplasmicEC 6.1.1.10; Methionyl-tRNA synthetase; MetRSHomo sapiens (human)
Serine hydroxymethyltransferase, cytosolicSHMT; EC 2.1.2.1; Glycine hydroxymethyltransferase; Serine methylaseHomo sapiens (human)
S-adenosylmethionine decarboxylase proenzymeAdoMetDC; SAMDC; EC 4.1.1.50Homo sapiens (human)
DNA (cytosine-5)-methyltransferase 1Dnmt1; EC 2.1.1.37; CXXC-type zinc finger protein 9; DNA methyltransferase HsaI; DNA MTase HsaI; M.HsaI; MCMTHomo sapiens (human)
Spermidine synthaseSPDSY; EC 2.5.1.16; Putrescine aminopropyltransferaseHomo sapiens (human)
S-adenosylmethionine synthase isoform type-2AdoMet synthase 2; EC 2.5.1.6; Methionine adenosyltransferase 2; MAT 2; Methionine adenosyltransferase II; MAT-IIHomo 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)
Cystathionine beta-synthaseEC 4.2.1.22; Beta-thionase; Serine sulfhydraseHomo sapiens (human)
Choline dehydrogenase, mitochondrialCDH; CHD; EC 1.1.99.1Homo sapiens (human)
L-amino-acid oxidaseLAAO; LAO; EC 1.4.3.2; EC 1.4.3.25; Interleukin-4-induced protein 1; IL4-induced protein 1; hIL4I1; Protein Fig-1; hFIG1Homo sapiens (human)
Methionine-R-sulfoxide reductase B3MsrB3; EC 1.8.4.12; EC 1.8.4.14Homo sapiens (human)
Methionine-R-sulfoxide reductase B2, mitochondrialMsrB2; EC 1.8.4.12; EC 1.8.4.14Homo sapiens (human)
Methylenetetrahydrofolate reductaseEC 1.5.1.20Homo sapiens (human)
Methionine adenosyltransferase 2 subunit betaMethionine adenosyltransferase II beta; MAT II beta; Putative dTDP-4-keto-6-deoxy-D-glucose 4-reductaseHomo sapiens (human)

Compounds (28)

CompoundDescription
Orthophosphate
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)
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
cholineA basic constituent of lecithin that is found in many plants and animal organs. It is important as a precursor of acetylcholine, as a methyl donor in various metabolic processes, and in lipid metabolism.
NADH
cysteineA thiol-containing non-essential amino acid that is oxidized to form CYSTINE.
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.
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.
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.
sarcosineAn amino acid intermediate in the metabolism of choline.
spermidineA polyamine formed from putrescine. It is found in almost all tissues in association with nucleic acids. It is found as a cation at all pH values, and is thought to help stabilize some membranes and nucleic acid structures. It is a precursor of spermine.
homoserine
5'-methylthioadenosinestructure
s-adenosyl-3-methylthiopropylaminedecarboxylated S-adenosylmethionine
2-keto-4-methylthiobutyric acidRN given refers to parent cpd; structure
alpha-ketobutyric acidRN given refers to parent cpd; structure
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)
serineA non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from GLYCINE or THREONINE. It is involved in the biosynthesis of PURINES; PYRIMIDINES; and other amino acids.
dimethylglycinemetabolic product of calcium pangamate; mutagen when mixed with nitrite; RN given refers to parent cpd
methionine sulfoxideRN given refers to cpd without isomeric designation
putrescineA toxic diamine formed by putrefaction from the decarboxylation of arginine and ornithine.
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)
pyrophosphate