aciduria — TargetMol Chemicals

Cat No.	Product Name	Synonyms	Targets
T4766	3-Methylglutaric acid		Endogenous Metabolite
	Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239 ). Methylglutaric acid is als...		Endogenous Metabolite
T19340	Glutarylcarnitine	O-glutaroyl-L-carnitine	Endogenous Metabolite
	Glutarylcarnitine (O-glutaroyl-L-carnitine) is a diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.		Endogenous Metabolite
T5305	3-Methylbut-2-enoic acid	3,3-Dimethylacrylic Acid,Senecioic acid,3-Methylcrotonic Acid	Others , Endogenous Metabolite
	3-Methylbut-2-enoic acid (Senecioic acid) appears in the urine of patients with 3-Methylcrotonic aciduria, which is a disorder characterized by urine that contains increased amounts of 3-methylcrotonic acid.		Others , Endogenous Metabolite
T5550	2-Hydroxy-2-methylbutanoic acid		Others , Endogenous Metabolite
	2-Hydroxy-2-methylbutyric acid is an unusual metabolite found in the urine of patients with 2-hydroxyglutaric aciduria and maple syrup urine disease.		Others , Endogenous Metabolite
T4861	Trans-Aconitic acid		ATPase , Endogenous Metabolite
	Trans-Aconitic acid is normally present in normal human urine, and it has been suggested that is present in larger amounts with Reye's syndrome and organic aciduria. trans-Aconitic acid is a substrate of enzyme trans-aco...		ATPase , Endogenous Metabolite
T5246	Trans-2-butene-1,4-dicarboxylic acid	trans-2-Butene-1,4-dicarboxylic Acid,3-Hexenedioic Acid,trans-3-Hexenedioic Acid,β-Hydromuconic Acid	Others , Endogenous Metabolite
	Trans-2-butene-1,4-dicarboxylic acid (3-Hexenedioic Acid) is a normal human unsaturated dicarboxylic acid metabolite with increased excretion in patients with Dicarboxylic aciduria caused by fatty acid metabolism disorde...		Others , Endogenous Metabolite
T5273	2-Hydroxy-3-methylbutanoic acid	2-Hydroxy-3-methylbutyric acid,2-Hydroxyisovaleric Acid,(±)-2-Hydroxyisopentanoic Acid	Others , Endogenous Metabolite
	2-Hydroxy-3-methylbutanoic acid ((±)-2-Hydroxyisopentanoic Acid) is a metabolite found in the urine of patients with phenylketonuria, methylmalonic acidemia, propionic acidemia, 3-ketothiolase deficiency, isovaleric acid...		Others , Endogenous Metabolite
T5232	3-Methyl-2-oxobutanoic acid	2-Oxoisovaleric acid,alpha-Ketoisovaleric acid	Others , Endogenous Metabolite
	3-Methyl-2-oxobutanoic acid (2-Oxoisovaleric acid) is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 3-Methyl-2-oxobutanoic acid is a neurotoxin, an acidogen, and a metabo...		Others , Endogenous Metabolite
T4880	L-Dihydroorotic acid		Others , Endogenous Metabolite
	L-Dihydroorotic acid, also known as (S)-4,5-dihydroorotate or dihydro-L-orotate, belongs to the class of organic compounds known as alpha amino acids and derivatives. Within the cell, L-dihydroorotic acid is primarily lo...		Others , Endogenous Metabolite
T4901	5'-Guanylic acid disodium salt	Guanosine 5'-monophosphate disodium salt,GMP-5	Others , Endogenous Metabolite
	5'-Guanylic acid disodium salt (GMP-5) belongs to the class of organic compounds known as purine ribonucleoside monophosphates. In particular, L-Glutamic acid and guanosine monophosphate can be biosynthesized from xanthy...		Others , Endogenous Metabolite
T19289	DL-Glyceric Acid	Glyceric Acid (20% in Water,ca.2 mol/L)	Others
	DL-glyceric acid is a compound that is excessively secreted in the urine of patients with D-glyceric aciduria.		Others
T73752	Glutarylcarnitine lithium
	Glutarylcarnitine lithium, a key diagnostic metabolite for malonic aciduria and glutaric aciduria type I, is routinely monitored in the majority of newborn screening programs utilizing tandem mass spectrometry [1].
T19346	5'-Guanylic acid	5'-guanosine monophosphate,5'-GMP	Endogenous Metabolite
	5'-Guanylic acid (5'-guanosine monophosphate) is involved in several metabolic disorders, including AICA-ribosiduria pathway, adenosine deaminase deficiency,adenine phosphoribosyltransferase deficiency (aprt), and the 2-...		Endogenous Metabolite
T38954	L-Glyceric acid sodium
	L-Glyceric acid sodium is a urinary metabolite predominantly found in individuals with the rare inherited metabolic disorder, L-glyceric aciduria. This compound serves as a diagnostic tool for identifying primary hyperox...
T78071	3-Methylglutaconic acid	β-Methylglutaconic acid	GABA Receptor
	3-Methylglutaconic acid, a primary metabolite accumulating in 3-Methylglutaconic aciduria (MGTA), induces lipid and protein oxidation, while diminishing non-enzymatic antioxidant defenses in cerebral cortex supernatants,...		GABA Receptor
T35698	Octanoic Acid-13C
	Octanoic acid-13C is intended for use as an internal standard for the quantification of octanoic acid by GC- or LC-MS. Octanoic acid is a medium-chain saturated fatty acid. It has been found in Teleme cheeses made from g...
T36055	Nitisinone-13C6	Nitisinone-13C6
	Nitisinone-13C6is intended for use as an internal standard for the quantification of nitisinone by GC- or LC-MS. Nitisinone is an inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD), which converts 4-hydroxyphenylpyr...