Page last updated: 2024-10-06

Non-Ketotic Hyperglycinemia

Proteins (24)

ProteinSynonymsTaxonomy
Serine--pyruvate aminotransferaseSPT; EC 2.6.1.51; Alanine--glyoxylate aminotransferase; AGT; 2.6.1.44Homo sapiens (human)
Cystathionine gamma-lyaseEC 4.4.1.1; Cysteine-protein sulfhydrase; Gamma-cystathionaseHomo sapiens (human)
Serine hydroxymethyltransferase, mitochondrialSHMT; EC 2.1.2.1; Glycine hydroxymethyltransferase; Serine methylaseHomo sapiens (human)
Amine oxidase [flavin-containing] AEC 1.4.3.4; Monoamine oxidase type A; MAO-AHomo sapiens (human)
Serine hydroxymethyltransferase, cytosolicSHMT; EC 2.1.2.1; Glycine hydroxymethyltransferase; Serine methylaseHomo sapiens (human)
Serine racemaseEC 5.1.1.18; D-serine ammonia-lyase; D-serine dehydratase; 4.3.1.18; L-serine ammonia-lyase; L-serine dehydratase; 4.3.1.17Homo sapiens (human)
Aldehyde dehydrogenase, mitochondrialEC 1.2.1.3; ALDH class 2; ALDH-E2; ALDHIHomo sapiens (human)
D-3-phosphoglycerate dehydrogenase3-PGDH; EC 1.1.1.95; 2-oxoglutarate reductase; 1.1.1.399; Malate dehydrogenase; 1.1.1.37Homo sapiens (human)
Serine--tRNA ligase, cytoplasmicEC 6.1.1.11; Seryl-tRNA synthetase; SerRS; Seryl-tRNA(Ser/Sec) synthetaseHomo sapiens (human)
Guanidinoacetate N-methyltransferaseEC 2.1.1.2Homo sapiens (human)
Glycine N-methyltransferaseEC 2.1.1.20Homo sapiens (human)
Phosphoserine phosphatasePSP; PSPase; EC 3.1.3.3; L-3-phosphoserine phosphatase; O-phosphoserine phosphohydrolaseHomo sapiens (human)
Glycine--tRNA ligaseEC 6.1.1.14; Diadenosine tetraphosphate synthetase; Ap4A synthetase; 2.7.7.-; Glycyl-tRNA synthetase; GlyRS; Glycyl-tRNA synthetase 1Homo sapiens (human)
L-serine dehydratase/L-threonine deaminaseSDH; EC 4.3.1.17; L-serine deaminase; L-threonine dehydratase; TDH; 4.3.1.19Homo sapiens (human)
Dihydrolipoyl dehydrogenase, mitochondrialEC 1.8.1.4; Dihydrolipoamide dehydrogenase; Glycine cleavage system L proteinHomo sapiens (human)
Phosphoserine aminotransferaseEC 2.6.1.52; Phosphohydroxythreonine aminotransferase; PSATHomo sapiens (human)
5-aminolevulinate synthase, nonspecific, mitochondrialALAS-H; EC 2.3.1.37; 5-aminolevulinic acid synthase 1; Delta-ALA synthase 1; Delta-aminolevulinate synthase 1Homo sapiens (human)
Glycerate kinaseEC 2.7.1.31; HBeAg-binding protein 4Homo sapiens (human)
Sarcosine dehydrogenase, mitochondrialSarDH; EC 1.5.8.3; BPR-2Homo sapiens (human)
Dimethylglycine dehydrogenase, mitochondrialEC 1.5.8.4; ME2GLYDHHomo sapiens (human)
2-amino-3-ketobutyrate coenzyme A ligase, mitochondrialAKB ligase; EC 2.3.1.29; Aminoacetone synthase; Glycine acetyltransferaseHomo sapiens (human)
Glycine dehydrogenase (decarboxylating), mitochondrialEC 1.4.4.2; Glycine cleavage system P protein; Glycine decarboxylase; Glycine dehydrogenase (aminomethyl-transferring)Homo sapiens (human)
Aminomethyltransferase, mitochondrialEC 2.1.2.10; Glycine cleavage system T protein; GCVTHomo sapiens (human)
Glycine amidinotransferase, mitochondrialEC 2.1.4.1; L-arginine:glycine amidinotransferase; TransamidinaseHomo sapiens (human)

Compounds (40)

CompoundDescription
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)
Orthophosphate
aminolevulinic acidA compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS.
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.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
creatineAn amino acid that occurs in vertebrate tissues and in urine. In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as CREATININE in the urine.
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 diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
berlitionantioxidant preparation containing alpha-lipoic acid, used in the neuroprotective therapy of chronic brain ischemia for correction of free-radical processes
methionineA sulfur-containing essential L-amino acid that is important in many body functions.
formaldehydeA highly reactive aldehyde gas formed by oxidation or incomplete combustion of hydrocarbons. In solution, it has a wide range of uses: in the manufacture of resins and textiles, as a disinfectant, and as a laboratory fixative or preservative. Formaldehyde solution (formalin) is considered a hazardous compound, and its vapor toxic. (From Reynolds, Martindale The Extra Pharmacopoeia, 30th ed, p717)
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.
Coenzyme A
phosphohydroxypyruvic acid
sarcosineAn amino acid intermediate in the metabolism of choline.
aminoacetoneRN given refers to parent cpd
dihydrolipoic acidRN given refers to parent cpd without isomeric designation
glyceric acidfound in urine of patient with D-glyceric acidemia & hyperglycinaemia; RN given refers to parent cpd without isomeric designation
2-amino-3-ketobutyrateunstable intermediate in threonine dehydrogenase-initiated pathway for threonine utilization; structure given in first source
alpha-ketoglutaric acid
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)
alanineA non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.
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
3-phosphoglycerate
glyoxylic acidRN given refers to parent cpd
pyruvaldehydeAn organic compound used often as a reagent in organic synthesis, as a flavoring agent, and in tanning. It has been demonstrated as an intermediate in the metabolism of acetone and its derivatives in isolated cell preparations, in various culture media, and in vivo in certain animals.
orotidylic acidstructure
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)
acetyl coenzyme aAcetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
pyrophosphate
hydroxypyruvic acidRN given refers to parent cpd